REPUBLIC OF ZAMBIA

MINISTRY OF HEALTH

ZAMBIA
PAEDIATRIC
PROTOCOLS

First Edition | 2020

TABLE OF CONTENTS
FLUID AND ELECTROLYTE REQUIREMENTS .......................................... 7
FLUID AND ELECTROLYTES .................................................................. 7
FLUID AND ELECTROLYTE THERAPY-MAINTENANCE .......................7
HYPONATRAEMIA ................................................................................7
HYPERNATRAEMIA ............................................................................... 9
HYPERKALAEMIA................................................................................11
HYPOKALAEMIA.................................................................................16
HYPOCALCEMIA ...............................................................................17
APPROACH TO INTERPRETATION OF ARTERIAL BLOOD GASES
(ABGS).................................................................................................20
MANAGEMENT OF SHOCK ..............................................................26
ASPIRIN ................................................................................................36
ORGANOPHOSPHATE POISONING.................................................38
HYDROCARBONS POISONING ........................................................39
IRON OVERDOSE ...............................................................................39
PARAQUAT POISONING ...................................................................41
DIABETIC KETOACIDOSIS (DKA) ......................................................43
ADRENAL CRISIS ................................................................................47
CARDIOVASCULAR SYSTEM ............................................................. 50
CONGESTIVE CARDIAC FAILURE (CCF) .........................................50
ACUTE RHEUMATIC FEVER ................................................................53
RHEUMATIC HEART DISEASE .............................................................57
INFECTIVE ENDOCARDITIS ................................................................59
SYSTEMIC HYPERTENSION .................................................................62
CYANOTIC SPELL ...............................................................................69
THE RESPIRATORY SYSTEM ................................................................ 74
CHILD WITH STRIDOR .........................................................................74
VIRAL CROUP (LARYNGOTRACHEOBRONCHITIS) ........................74

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 ACUTE EPIGLOTTITIS ...........................................................................78
FOREIGN BODY INHALATION ...........................................................79
THE WHEEZING CHILD .......................................................................82
BRONCHIOLITIS ..................................................................................88
CONGENITAL CARDIAC DISEASE....................................................93
ACUTE EXACERBATION OF ASTHMA.............................................105
PNEUMONIA .....................................................................................109
CENTRAL NERVOUS SYSTEM ........................................................... 113
COMA ...............................................................................................113
INTRACRANIAL PRESSURE ...............................................................119
SEIZURES ............................................................................................122
STATUS EPILEPTICUS .........................................................................128
FEBRILE SEIZURES ..............................................................................135
PAEDIATRIC STROKE ........................................................................139
GENITOURINARY SYSTEM ............................................................... 144
URINARY TRACT INFECTION ...........................................................144
HAEMATURIA ....................................................................................146
NEPHROTIC SYNDROME .................................................................150
ACUTE GLOMERULONEPHRITIS ......................................................153
ACUTE KIDNEY INJURY ....................................................................157
GASTROINTESTINAL SYSTEM ........................................................... 163
ACUTE DIARRHOEAL DISEASE ........................................................163
PERSISTENT DIARRHOEA (PD) .........................................................169
ACUTE UPPER GI BLEEDING (OLD) ................................................172
FUNCTIONAL CONSTIPATION ........................................................174
ACUTE HEPATIC FAILURE (OLD) .....................................................177
INTESTINAL OBSTRUCTION ..............................................................180
INTUSSUSCEPTION ............................................................................183
INFANTILE COLIC .............................................................................185

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 GASTROESOPHAGEAL REFLUX DISEASE (GERD) .........................188
PROLONGED JAUNDICE ................................................................191
PEPTIC ULCER DISEASE ....................................................................195
VOMITING .........................................................................................197
HYPERTROPHIC PYLORIC STENOSIS...............................................200
HAEMATO-ONCOLOGY ................................................................. 203
ANAEMIA ..........................................................................................203
SICKLE CELL DISESASE – CVA.........................................................207
HAEMOPHILIA ..................................................................................212
IMMUNE THROMBOCYTOPENIA (ITP) ............................................216
APLASTIC ANEMIA ...........................................................................221
APPROACH TO DIAGNOSIS AND INVESTIGATION OF COMMON
MALIGNANCIES ...............................................................................227
MANAGEMENT OF PAINFUL CRISIS ...............................................231
INFECTIOUS DISEASE ....................................................................... 233
MALARIA ...........................................................................................233
TYPHOID (ENTERIC FEVER) - Dr Mulenga .....................................237
SCHISTOSOMIASIS............................................................................239
TUBERCULOSIS IN CHILDREN ..........................................................242
POST EXPOSURE PROPHYLAXIS - OCCUPATIONAL HIV EXPOSURE
...........................................................................................................248
ELIMINATION OF MOTHER TO CHILD HIV TRANSMISSION - EMTCT
...........................................................................................................249
ENDOCRINE..................................................................................... 252
DISORDER OF SEXUAL DIFFERENTIATION (DSD) ...........................252
RICKETS..............................................................................................257
PRECOCIOUS PUBERTY (PP) ...........................................................260
HYPOGLYCAEMIA IN CHILDREN AND ADOLESCENTS ...............265
GRAVE’S DISEASE (GD) ..................................................................271
HYPOTHYROIDISM ...........................................................................274

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NEONATOLOGY .............................................................................. 279
HYPOXIC ISCHAEMIC ENCEPHALOPATHY (HIE) .........................279
PREMATURITY ....................................................................................286
RESPIRATORY DISTRESS SYNDROME ..............................................290
NECROTISING ENTEROCOLITIS (NEC) ...........................................291
SEPSIS IN NEONATES ........................................................................294
NEONATAL SEIZURES........................................................................299
NEONATAL JAUNDICE ....................................................................303
PROLONGED NEONATAL JAUNDICE............................................310

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Forewo rd

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Ack nowle dge me nt

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FLUID AND ELECTROLYTE REQUIREMENTS

FLUID AND ELECTROLYTES

The body loses fluids through urine, insensible loses (respiration,
skin, stools) and intrinsic losses (by-product of metabolism).

FLUID AND ELECTROLYTE THERAPY-MAINTENANCE

The goal of maintenance therapy is to replace fluid and
electrolyte needed by the average child with normal renal
function over a 24-hour period.
Calculating maintenance fluids: Holliday-Segar method.

Fluid per day Rate per hour

1st 10kg 100 mL/kg/day 4 mL/kg/hr

Next 10kg 50 mL/kg/day 2 mL/kg/hr
For every additional kg 20 mL/kg/day 1 mL/kg/hr

Maintenance electrolyte requirements in children are:

 2-3 mmol/kg/day of sodium,
 1-2 mmol/kg/day of potassium
 3-5 mmol/kg/day of chloride

HYPONATRAEMIA
Hyponatraemia is serum sodium (Na) concentration of less than
135 mmol/L. Many patients presenting with acute neurological
symptoms may have unrecognised hyponatraemia.

Causes
 Hypotonic fluid hydration
 Diarrhoea & vomiting
 Acute & chronic renal failure
 Hypothyroidism; adrenal insufficiency; SIADH
 Cirrhosis; congestive cardiac failure; nephrotic syndrome
 Ascites; effusions; pancreatitis
 Drugs - diuretics

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Clinical features
Symptoms occur when serum Na levels fall below 125 mmol/L. If
the changes are chronic, the patient may be asymptomatic.
These are:

 Early signs: anorexia, headache, nausea & vomiting

 Hypo/hypertension, muscle weakness & cramps,
 Bizarre behaviour, hallucinations, seizures,
decorticate/decerebrate posture, coma

Investigations
 Serum Na+; serum osmolality; Urea & creatinine; Urine
osmolality & Na+
 Aldosterone, cortisol, free T4 & TSH, ACTH & ADH levels
 Imaging studies for underlying cause – U/S & CT scans (head,
abdomen)

Management
Correction of hyponatraemia should be slow, not exceeding 8
mmol/L/day.
 With neurologic symptoms, raise the serum Na concentration
by giving doses of 1-2 ml/Kg of 3% saline until symptoms
resolve. Symptoms typically resolve with a rise in sodium of 3-7
mmol/L
 Hypovolaemic hyponatraemia - correct volume depletion
with normal saline, then restriction of fluids to two-thirds (or
less) of the volume needed for maintenance
 Normovoalemic hyponatraemia due to SIADH include fluid
restriction to two-thirds (or less) of the volume needed for
maintenance using normal saline; or the use of 3% NaCl, and
IV administration of furosemide
 Hypervolemic hyponatraemia - restrict fluids and administer
3% NaCl to stop the symptoms
 Treat the underlying cause

NOTE: Osmotic Demyelination Syndrome: Brain injury due to too-

rapid correction of chronic hyponatremia is often associated
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with delayed neurological deterioration (onset of symptoms 1 to
4 days after serum Na is increased by > 12 mmol/L in less than 24
hours).

HYPERNATRAEMIA
Hypernatraemia is a serum sodium level greater than 145
mmol/L.

Causes
Excessive Sodium Water Deficit Water and Sodium
Deficit
Improperly mixed Nephrogenic diabetes
Diarrhoea
formula insipidus
Cow’s milk (UHT, Fresh Central diabetes
Emesis
milk) insipidus
Excess sodium Increased insensible
Burns
bicarbonate losses
Chronic kidney
IV hypertonic saline Inadequate intake
disease
Osmotic diuresis

Clinical Features
 Irritability
 Restlessness
 Lethargy
 High pitched cry
 Hyperpnoea
 Cerebral haemorrhage
 Seizures
 Coma
 Stroke

Complications
 Dural sinus thrombosis
 Peripheral thrombosis
 Renal vein thrombosis

Management

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 Priority is restoration of intravascular volume with isotonic fluid (slow infusion
of normal saline).
Firstly, the water deficit is calculated as follows:
Water deficit (in Litres): = Body weight × 0.6 (1-145/ [current sodium] )
Replacement fluid is then calculated as follows:
Replacement volume (in L)
= TBW deficit X [1 ÷ 1 - (Na concentration in replacement fluid in mmol/L ÷
154 mmol/L)]

The volume calculated must be replaced slowly over 48-72

hours.
For example:

A 10kg child arrives with a history of dehydration. His labs reveal

a Sodium 160 mmol/l.
Water Deficit: 10 x 0.6 x (1-145/160) = 0.6 litres
Fluid available is ½ Strength Darrow’s: Sodium content
in Darrow’s = 61 mmol/L.

Therefore:-
replacement volume: 0.6 litres x [1/1- (61/154)] =1 litre
This volume of fluid should be given over 48-72 hours in addition
to the daily maintenance fluid requirement.

Please monitor sodium six hourly. Aim at not dropping sodium

by more than 0.5 mmol/hour or a total of 12 mol in any 24
hour period.

Other measures
 Severe cases (>200 mmol/L) may require dialysis with a high
glucose-low sodium dialysate.
 Hyperglycaemia resulting from hypernatremia is not usually
treated with insulin as rapid lowering of glucose may
precipitate cerebral oedema.
 The underlying cause should be treated whenever possible

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 Central diabetes insipidus should be treated with
desmopressin

HYPERKALAEMIA
Hyperkalaemia is defined as serum potassium (K) level of more
than 5mmol/L or 6mmol/L in neonates.

Classificatio n of H ype rkalaemia

Severe Moderate Mild
K+ >7.0 mmol/L or at K+ 6-7 mmol/L K+ >5.5 mmol/L
risk of increasing
and/or
Patient symptomatic Asymptomatic Asymptomatic
and/or
ECG disturbance: ECG may be normal Normal ECG

Causes
Increased Intake Decreased Excretion Drugs
Intravenous or oral Renal failure Angiotensin converting
enzyme inhibitors
Blood transfusions Urinary tract Angiotensin II blockers
obstruction
Renal transplant Potassium sparing
diuretics
Renal tubular disease Nonsteroidal anti-
inflammatory drugs

Clinical Features
 Paraesthesia
 Weakness
 Tingling
 ECG changes peaked T waves, wide P-R interval, flattening p
wave, widening QRS complex
 Ventricular fibrillation
 Asystole

Management
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Aims
 Stabilise the heart to prevent life threatening arrhythmias
 Remove potassium from the body
 The flow chart (figure xx) demonstrates the step by step
approach

Hyperkalaemia K+ ≥ 5.5

Stop all K+ supplementation.

Stop medication causing hyperK+
Cardiac monitoring

Exclude pseudohyperkalaemia
(recheck with venous sample)

Patient unstable or Patient asymptomatic and

Patient Asymptomatic
Symptomatic or normal ECG and K+ >5.5,
and Norma ECG and K+
clearly abnormal ECG ≤6.0 mmol/L.
>6, ≤7 mmol/L
or K+ >7mmol/L

Salbutamol NEB. Consider if treatment

Discuss dialysis. Insulin/Glucose perfusion IV. necessary.
Consider transfer ± Resonium PR or oral. ± Salbutamol NEB.
to tertiary centre. ± Bicarbonate IV if metabolic
acidosis.
± Polystyrene sulfonate
PR or oral

Calcium IV.
Salbutamol NEB. ± Bicarbonate IV if
Insulin / Glucose IV. metabolic acidosis.
Bicarbonate IV if metabolic
acidosis.
± Resonium PR or oral.

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13
 Drug Dosage Onset and Expected Actions Mechanism Considerations
Calcium 0.5mL/kg (0.11mmol/kg) in neonates and Onset of Action: <3 minutes, Stabilises the NOT to be given
10% calcium gluconate children <12 years. Max dose 4.5 mmol should see normalisation of heart simultaneously with
(Approx. 225 (20mL) of 10% calcium gluconate OR ECG. If not: repeat dose bicarbonate
micromol/mL) 0.1-0.2 ml/kg slow IV injection (as above) (twice) NOT to be given if
Calcium Chloride 10% (Max: 10ml) of Calcium Chloride 10% Duration: About 30 minutes Digitalis toxicity
Soluble insulin/ Glucose Average dosage: 0.3-0.6 IU/kg/hr in Onset of Action: 15 min Shifts potassium To be given at the
neonates, 0.05-0.2IU/kg/hr in children Duration: peak 60 minutes, 2- into the cells same time.
1month and older; 5-10ml/Kg of D10% 3hours
If severe hyperkalaemia: Closely monitor
• Dextrose 10%: 5ml/kg IV bolus (if no glucose every 30-60
hyponatremia) minutes
• Insulin short action: 0.1 U/kg IV bolus (Max
10 units)
Then followed by infusion insulin/glucose
(see below)
If moderate hyperkalaemia:
• Dextrose 10% IV at maintenance with
0.9% sodium chloride (normal saline)
• Insulin short action infusion: 0.1 U/kg/h IV

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 Drug Dosage Onset and Expected Actions Mechanism Considerations
8.4% NaHCO3 (IV) Severe hyperkalaemia and metabolic Onset of Action: 30-60 minutes Shifts potassium into In metabolic acidosis,
acidosis Duration: 2-3 hours the cells only.
Sodium Bicarbonate 8.4% 1mmol/ml: 1-
3ml/kg IV over 5 minutes
Mild to moderate hyperkalaemia and
metabolic acidosis:
Sodium Bicarbonate 8.4% 1mmol/ml:
1ml/kg in slow IV infusion over 30 minutes
Salbutamol (nebulised or < 25kg: 2.5 mg nebulised q 1-2h Onset of Action: 30 minutes Shifts potassium into Reduces intravascular K+
IV) More than 25kg: 5mg nebulised (max 10- Duration: 2-3 hours cells of 0.5-1.5mmol/L
20mg) q 1-2h
Resonium Mild effect, multiple doses necessary, Onset of Action: 1-hour PR, 4- Potassium excretion Mild effect, multiple doses
may be used as long term agent 6hours oral, should reduce through gut necessary, may be used
Polystyrene sulfonate (Resonium) 0.3- intravascular K+ of 0.5-1 mmol/L as long term agent.
1g/kg q 6h (Max 15-30g) Per Rectal (PR) Duration: variable NOT to be used if ileus,
or oral (with lactulose) recent abdominal surgery,
perforation,
hypernatremia

Dialysis Transfer to tertiary care centre Rapid- haemodialysis Removes potassium Organise with local
Slower- peritoneal Paediatric renal or
intensive care team.

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Consider cons ultatio n with loc al paediatric team:
 Any child with moderate or severe hyperkalemia
 Underlying medical cause – e.g., renal abnormalities

When to consider transfer to tertiary ce ntre :

 Any child with severe hyperkalemia
 Any child requiring dialysis
 Child requiring care beyond the comfort level of the hospital.

HYPOKALAEMIA
This a serum potassium level < 3mmol/L.

Causes
Decreased Extra-renal Renal losses Drugs
intake losses
Anorexia Diarrhoea Renal tubular Amphotericin
nervosa acidosis
Laxative Diabetic Cisplatin
abuse ketoacidosis
Sweating Interstitial nephritis Aminoglycosides
Uretero- Loop and thiazide
sigmoidostomy diuretics
Cystic fibrosis

Clinical features
 Muscle weakness and cramps
 Paralysis
 Heart block
 Paralytic ileus
 Polyuria
 Polydipsia
 ECG features: Flattened T waves, depressed ST segment, U
wave

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 Supraventricular tachycardia
 Ventricular tachycardia

Management
Oral Slow-K and Potassium chloride is preferred.
Intravenous potassium may be given at a dose of 0.5-1mmol/Kg
usually given over one hour. Intravenous potassium should be
used cautiously, with close monitoring of electrolytes, because of
the risk of hyperkalaemia.

HYPOCALCEMIA
Hypocalcaemia is a total serum calcium concentration < 8.8
mg/dL (< 2.20 mmol/L) in the presence of normal plasma protein
concentrations or a serum ionized calcium concentration < 4.7
mg/Dl (< 1.17 mmol/L).

Causes :
 Hypoparathyroidism
 Pseudohypoparathyroidism
 Vitamin D deficiency and dependency
 Renal disease
 Magnesium depletion
 Acute pancreatitis
 Hypoproteinaemia (asymptomatic)
 Persistent hypocalcaemia and hypophosphatemia correction
of moderate to severe hyperparathyroidism
 Septic shock
 Hyperphosphatemia
 Drugs including anticonvulsants
(e.g., phenytoin, phenobarbital) and rifampicin,
 Transfusion of > 10 units of citrate-anticoagulated blood

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 Use of radiocontrast agents containing the divalent ion-
chelating agent
 Infusion of gadolinium

Clinical features
Tetany characteristically results from severe hypocalcaemia but
can result from reduction in the ionized fraction of serum calcium
without marked hypocalcaemia, as occurs in severe alkalosis. It
is characterized by the following:
 Sensory symptoms consisting of paraesthesia of the lips,
tongue, fingers, and feet
 Carpopedal spasm, which may be prolonged and painful
 Generalized muscle aching
 Spasm of facial musculature
 Seizures
 Twitching
 Cramping
 Laryngospasm, a rare initial manifestation

Diagnosis
 Estimation or measurement of ionized calcium
 Measurement of serum magnesium, PTH, phosphate, alkaline
phosphatase (ALP), and vitamin D concentrations
 Urine calcium, magnesium, and phosphate.

Treatme nt
 Oral calcium therapy (Calcium gluconate, Calcium
carbonate) is preferred in asymptomatic patients and as
follow-up treatment after intravenous (IV) calcium therapy
 IV calcium gluconate for tetany and patients having seizures,
critically ill, and pre-operatives.
 Oral calcium for postoperative hypoparathyroidism
 Oral calcium and vitamin D for chronic hypercalcemia
 Ensure that the patient is well hydrated and passing urine

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 Correction of hypomagnesemia is essential as
hypocalcaemia does not respond until the low magnesium
level is corrected.

Tetany
 IV Calcium gluconate should be given as follows:
o Give calcium gluconate 10 mL of 10% solution IV over 10
min; Calcium infusion drips should be started at 0.5
mg/kg/hr and increased to 2 mg/kg/hr as needed.
o Repeated boluses or a continuous infusion with 20 to 30
mL of 10% calcium gluconate in 1L of 5% D/W over the
next 12 to 24 to may be needed.
o Give bolus of 0.5mL/kg (0.11mmol/kg) of 10% solution in
neonates and children <12 years (Max dose 4.5 mmol
i.e. 20mL of 10% solution). For IV infusion dilute to at least
45 micromol/mL with D5% or 0.9% sodium chloride.
o Oral supplements (Calcium gluconate) may precipitate
NEC in neonates.

NOTE: (1) Infusions of calcium are hazardous in patients

receiving digoxin and should be given slowly and with
continuous ECG monitoring after checking for and correcting
hypokalaemia. (2) When tetany is associated with
hypomagnesemia, give a 10% magnesium sulphate solution (1
g/10 mL) IV, followed by oral magnesium salts (eg, magnesium
gluconate 500 to 1000 mg po tid).

Transient hypo parathy roidism

Give supplemental oral calcium: 1 to 2 g of elemental
calcium/day can give as calcium gluconate.

Chronic hypocalcae mia

Give oral calcium and/or vitamin D supplements: 1 to 2 g of
elemental calcium/day as calcium gluconate or calcium
carbonate.
In patients without renal failure, give vitamin D as a standard oral
supplement (e.g., cholecalciferol 800 IU once/day) with
adequate dietary or supplemental calcium and phosphate.

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In patients with renal failure, give calcitriol or another 1,25(OH)2D.

In hypoparathyroidism, give calcitriol, 0.5 to 2 mcg po once/day.

In Pseudohypoparathyroidism give calcitriol requires 1 to 3
mcg/day.
Monitor Serum calcium concentration weekly at then at 1- to 3-
mo intervals after calcium concentrations have stabilized as the
maintenance dose of calcitriol or its analogy,
dihydrocholecalciferol, usually decreases with time.

APPROACH TO INTERPRETATION OF ARTERIAL BLOOD GASES

(ABGS)
ABG interpretation is especially important in critically ill patients.
6-step approach below, is essential during the interpretation:
Step 1: Assess the internal consistency of the values using the
Henderson-Hasselbalch equation:
[H+] = 24(PaCO2)
[HCO3-]

If the pH and the [H+] are inconsistent, the ABG is probably not
valid.
Step 2: Is there alkalemia or acidaemia present?

pH < 7.35 acidaemia

pH > 7.45 alkalaemia

This is usually the primary disorder
Remember: an acidosis or alkalosis may be present even if the
pH is in the normal range (7.35 – 7.45)
You will need to check the PaCO2, HCO3- and anion gap
Step 3: Is the disturbance respiratory or metabolic? What is the
relationship between the direction of change in the pH and the
direction of change in the PaCO2?
Acidosis Respiratory pH ↓ PaCO2 ↑

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 Acidosis Metabolic& pH ↓ PaCO2 ↓
Alkalosis Respiratory pH ↑ PaCO2 ↓
Alkalosis Metabolic pH ↑ PaCO2 ↑

Step 4: Is there appropriate compensation for the primary

disturbance? Usually, compensation does not return the pH to
normal (7.35 – 7.45).
Disorder Expected compensation Correction
factor
Metabolic acidosis PaCO2 = (1.5 x [HCO3-]) +8 ±2
Acute respiratory acidosis Increase in ±3
[HCO3-]= ∆ PaCO2/10
Chronic respiratory Increase in
acidosis (3-5 days) [HCO3-] = 3.5(∆ PaCO2/10)
Metabolic alkalosis Increase in
PaCO2 = 40 + 0.6(∆HCO3-)
Acute respiratory alkalosis Decrease in
[HCO3-]= 2(∆ PaCO2/10)
Chronic respiratory Decrease in [HCO3-] = 5(∆
alkalosis PaCO2/10) to 7(∆ PaCO2/10)

Step 5: Calculate the anion gap (if a metabolic acidosis exists):

AG= [Na+]-( [Cl-] + [HCO3-] )-12 ± 2

 A normal anion gap is approximately 12 mmol/L.

 In patients has hypoalbuminemia, the AG is about 2.5 mmol/L
lower for each 1 g/dL decrease in the plasma albumin
concentration (for example, a patient with a plasma albumin
of 2.0 g/dL would have an AG of approximately 7mmol /L).
 If the anion gap is elevated, consider calculating the osmolal
gap in compatible clinical situations.
o Elevation in AG is not explained by an obvious case
(DKA, lactic acidosis, renal failure)
o Toxic ingestion is suspected
 OSM gap = measured OSM – (2[Na+] - glucose/18 – BUN/2.8
o The OSM gap should be < 10
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Step 6: If an increased anion gap is present, assess the
relationship between the increase in the anion gap and the
decrease in [HCO3-].
Assess the ratio of the change in the anion gap (∆AG) to the
change in [HCO3-] (∆[HCO3-]): ∆AG/∆[HCO3-]

This ratio should be between 1.0 and 2.0 if an uncomplicated

anion gap metabolic acidosis is present.
If this ratio falls outside of this range, then another metabolic
disorder is present:
 If ∆AG/∆[HCO3-] < 1.0, then a concurrent non-anion gap
metabolic acidosis is likely to be present.
 If ∆AG/∆[HCO3-] > 2.0, then a concurrent metabolic alkalosis
is likely to be present.

Characte ristics of acid-base dist urbances

Disorder pH Primary problem Compensation

Metabolic acidosis ↓ ↓ in HCO3- ↓ in PaCO2

Metabolic alkalosis ↑ ↑ in HCO3- ↑ in PaCO2
Respiratory acidosis ↓ ↑ in PaCO2 ↑ in [HCO3-]
Respiratory alkalosis ↑ ↓ in PaCO2 ↓ in [HCO3-]

Selected aetiologies of respiratory acidosis

 Airway obstruction
 CNS depression
 Sleep disordered breathing
 Neuromuscular impairment
 Ventilatory restriction
 Increased CO2 production: shivering, rigors, seizures,
malignant hyperthermia, hypermetabolism, increased intake
of carbohydrates
 Incorrect mechanical ventilation settings

Selected aetiologies of respiratory alkalosis

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 CNS stimulation: fever, pain, fear, anxiety, CVA, cerebral
oedema, brain trauma, brain tumour, CNS infection
 Hypoxemia or hypoxia: lung disease, profound anaemia, low
FiO2
 Stimulation of chest receptors: pulmonary oedema, pleural
effusion, pneumonia, pneumothorax, pulmonary embolus
 Drugs, hormones: salicylates, catecholamines,
medroxyprogesterone, progestins
 Pregnancy, liver disease, sepsis, hyperthyroidism
 Incorrect mechanical ventilation settings

Selected causes of metabolic alkalosis

 Hypovolemia with Cl- depletion
o GI loss of H+
 Vomiting, gastric suction, villous adenoma, diarrhoea
with chloride-rich fluid
o Renal loss H+
 Loop and thiazide diuretics, post-hypercapnia
(especially after institution of mechanical ventilation)
 Hypervolemia, Cl- expansion
o Renal loss of H+: oedematous states (heart failure,
cirrhosis, nephrotic syndrome), hyperaldosteronism,
hypercortisolism, excess ACTH, exogenous steroids,
hyperreninemia, severe hypokalaemia, renal artery
stenosis, bicarbonate administration

Selected aetiolo gies o f metabolic acidosis

 Elevated anion gap:
o Methanol intoxication
o Uraemia
o Ketoacidosis
o Paraldehyde toxicity
o Isoniazid
o Lactic acidosis
o Ethanol or ethylene glycol intoxication
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 o Salicylate intoxication

 Normal anion gap: will have increase in [Cl-]

o GI loss of HCO3- (Diarrhoea, ileostomy, proximal
colostomy)
o Renal loss of HCO3- (proximal RTA, acetazolamide),
Renal tubular disease (ATN, Chronic renal disease)

Selected mixed and complex acid-base disturbances

Disorder Characteristics Selected situations
Respiratory acidosis ↓in pH Cardiac arrest
with metabolic ↓ in HCO3 Intoxications
acidosis ↑ in PaCO2 Multi-organ failure
Respiratory alkalosis ↑in pH Cirrhosis with diuretics
with metabolic ↑ in HCO3- Pregnancy with vomiting
alkalosis ↓ in PaCO2 Over ventilation of COPD
Respiratory acidosis pH in normal COPD with diuretics, vomiting,
with metabolic range NG suction
alkalosis ↑ in PaCO2, Severe hypokalaemia
↑ in HCO3-

Respiratory alkalosis pH in normal Sepsis

with metabolic range Salicylate toxicity
acidosis ↓ in PaCO2 Renal failure with CHF or
↓ in HCO3 pneumonia
Advanced liver disease
Metabolic acidosis pH in normal Uraemia or ketoacidosis with
with metabolic range vomiting, NG suction, diuretics,
alkalosis HCO3- normal etc.

Treatme nt
Treatment is dependent on correctly identifying the acid-base
disorder and, whenever possible, repairing the underlying causal
process to maintain serum pH greater than 7.20.

Refere nces

24
1. Friedman A.L. Paediatric hydration therapy Historical
review and a new approach. Kidney
International (2005) 67, 380–388; doi:10.1111/j.1523-
1755.2005.00092.
2. Vellaichamy M. eMedicine. University of Kansas School
of Medicine, Wesley Medical Centre, Updated April 16,
2009
3. Rachel S. Pediatric fluids and electrolytes, Rutgers
University, Saint Barnabas Medical Centre, Sept 27, 2007
4. Kleigman, Behrman, Jenson, Stanton: Nelson Textbook of
Paediatrics. Volume 1. 18th Edition, 2007
5. Sidwell R and Thompson M. Concise Paediatrics.
Concise Paediatrics. 2nd Edition.
6. eMedicine. Hypernatremia. Paediatrics Cardiac Care
and Critical Care Medicine
7. Rose, B.D. and T.W. Post. Clinical physiology of acid-base
and electrolyte disorders, 5th ed. New York: McGraw Hill
Medical Publishing Division, c2001.
8. Fidkowski, C And J. Helstrom. Diagnosing metabolic
acidosis in the critically ill: bridging the anion gap,
Stewart and base excess methods. Can J
Anesth2009;56:247-256.
9. Adrogué, H.J. and N.E. Madias. Management of life-
threatening acid-base disorders—first of two parts. N
Engl J Med 1998;338:26-34.
10. Adrogué, H.J. and N.E. Madias. Management of life-
threatening acid-base disorders—second of two parts. N
Engl J Med 1998;338:107-111.
11. BNF for children 2014-15

25
EMERGENCY MEDICINE

MANAGEMENT OF SHOCK
Recognition of shock: Cold extremities with capillary refill time >3
second and weak and fast pulse.
Immediate goals [ABCDE]
 A: Stabilize the airway, ensure patent using airway adjunct
(e.g. airway positioning, chin tilt head lift, jaw thrust, guedel
airway)
 A: If severe angioedema, consider severe anaphylaxis as
cause
o Treat as per WHO guidelines: Pocketbook of Hospital
care of children page 108.
 B: Provide oxygen by mask (15L high flow) or nasal catheter if
mask unavailable (2 litre/min).
 C: Establishment of vascular access
o Two large bore peripheral intravenous catheters should
be established.
o If peripheral access is not readily available, intra-osseous
access should be established.
 C: Assess for malnutrition (wasting, peripheral oedema, skin
changes, z-score) and severe anaemia (pallor).
 D: Disability (AVPU – Alert/responsive to Voice/responsive to
Pain/Unresponsive) and Do Not Forget Glucose (do RBS and
treat appropriately).
 E: Exposure (body temperature, rash).
Weigh the child or estimate the child’s weight using formulas
below

 If <1 year old (kg) : (Age in months + 9) /2

 >1year old (kg) : (Age +4) x 2
 This does not substitute a child’s true weight – the child must
be weighed immediately once stable

26
See flow chart for management of shock on next page and
table for different types of shock.

27
Fig. xx Management of Shock Flow Chart

Diarrhoea and dehydration: WHO PLAN C: Use RL/NS

 Without SAM: 20mls/kg of RL/NS then WHO Plan C.
<1 year > 1 year
 With SAM: see SAM critical care pathway. First Over 1 Over 30
30mls/kg hour minutes
Recognition of shock Then Over 5 Over 2 ½
Severe Anaemia:
70mls/kg hours hours
 Cool peripheries  Urgent crossmatching and whole blood transfusion
Capillary refill > 3s
 Without SAM: 20mls/kg over 3 hours
 Weak and fast pulse ORS 5ml/kg/hour as soon as can
 Ensure immediate  With SAM: see SAM critical care pathway. drink. Regular re-assessment of
goals (ABCDE) met.  IV maintenance fluids whilst awaiting: as per fluid dehydration and treat as needed.
and electrolyte guideline

Other Children

 Without SAM: 10-20mls/kg of RL/NS then reassess

 With SAM: see SAM critical care pathway.
 Consider different causes of shock and treat
appropriately.

28
SAM = Severe Acute Malnutrition, RL = Ringers Lactate, NS = 0.9% Normal Saline, ORS = Oral Rehydration Salts.

29
FURTHER MANAGEMENT
 Continually reassess with thorough history and clinical examination.
o Start broad spectrum antibiotics within first hour (as per local
protocol).
o Maintain normothermia (temperature 36.5-37.5C): Keep warm /
give antipyretics
o If blood sugar < 3.0 correct with 10% dextrose 5ml/kg and
recheck after 15 minutes.
o Consider different types of shock (See next page) and treat
appropriately.
o Liaise with senior doctors if not improving
 Refractory shock
- If no response to 20mls/kg fluid bolus, carefully administer
second bolus of 20mls/kg.
- If no response to 40mls/kg fluid bolus, then assess for referral to
ICU/higher level hospital, prepare for intubation and start
inotropes with cardiac monitoring
 Unless the child is severely anaemic, whole blood transfusion is not
recommended for circulatory shock. Prepare to use inotropes as a first
line when not responding to fluid resuscitation.

30
TABLE XX. Types of shock

Type of shock Signs and symptoms Treatment

Hypovolaemic Increased HR, - Repeat boluses of 20
(reduced CO, reduced pulses, ml/kg crystalloid as
increased SVR) delayed CRT, indicated up to 40
hyperpnoea, dry skin, ml/kg in first hour.
sunken eyes, oliguria - Blood product as
indicated for acute
blood loss.
Septic Increased HR, normal -Repeat boluses of 20
(increased CO, to reduced BP, ml/kg crystalloid; may
increased SVR) reduced pulses, need up to 40ml/kg in
delayed capillary refill first hour.
time, hyperpnoea, - Consider colloid if
mental state changes, poor response to
third spacing, crystalloid.
oedema - Pharmacologic
support of BP with
dopamine or
norepinephrine.
Distributive Angioedema, rapid - Repeat boluses of 20
Anaphylaxis third spacing of fluids, ml/kg crystalloid as
(increased CO, reduced BP, indicated.
reduced SVR) respiratory distress -Pharmacologic
support of SVR with
norepinephrine or
phenylephrine.
Distributive Reduced BP with -Pharmacologic
Spinal cord injury normal HR, paralysis support of SVR with
(normal CO, reduced with loss of vascular norepenephrine or
SVR) tone phenylephrine.
-Fluid resuscitation as
indicated by clinical
status and associated
injuries.
Cardiogenic Normal to increased -Pharmacologic
(reduced CO, normal HR, reduced pulses, support of CO with
to increased SVR) delayed CRT, oliguria, dobutamine and
JVD, hepatomegaly dopamine.
BP normal until late in -Judicious fluid
course replacement as
indicated clinically.

31
CO = cardiac output, SVR = systemic vascular resistance, HR =
heart rate, BP = blood pressure, CRT = capillary refill time, MS =
mental status, JVD = jugular venous distension
FORMULAE FOR ADMINISTRATION OF VASOACTIVE MEDICATIONS
Drug Dose ranges Infusion Preparation Infusion rate
Dopamine Dopamine Body weight in kg x 6 1 ml/h = 1
Dobutamine (3 to 20 = Amount of drug mcg/kg per
mcg/kg/min) (mg) minute
to be added to total (Example: to
volume of 100 ml IV deliver 10
Dobutamine (1 to 20 fluid mcg/kg per
mcg/kg/min) minute, run
infusion at 10
ml/h)
Epinephrine Epinephrine (0.01 to Body weight in kg x 1 ml/h = 0.1
Norepinephrine 1.0 mcg/kg/min) 0.6 = Amount of drug mcg/kg per
Milrinone (mg) minute
to be added to total (Example: to
Norepinephrine volume of 100 ml IV deliver 0.3
(0.01 to 1.0 fluid mcg/kg per
mcg/kg/min minute, run
infusion at
3ml/h)
NOTE: Dopamine and epinephrine can be given peripherally at
a maximum dose of range of …. (need to check dose).

References

Christian A McKeirnan and Stephen A Leiberman.

Circulatory shock in children: An overview. Pediatr. Rev 2005

Stephen M. Schexnayder. Pediatric septic shock;

Pediatr.Rev.1999
Updated Guideline: Paedaitric Emergency Triage,
assessment and treatment. Geneva: World Health
Organization; 2016.
WHO 2013. Pocket book for Hospital care for children
(Second edition). Geneva: World Health Organisation; 2013.

32
ANAPHYLAXIS REACTION PROTOC OL
Definition
Anaphylaxis is a severe allergic reaction that can be life
threatening because of airway compromise and circulatory
shock. Common causes include: Antibiotics, vaccines, blood
transfusion and certain foods (e.g. nuts) and medications. There
are associations with other allergic disorders e.g. asthma,
eczema, family history.

Management
Manage via the ABCDE approach (see shock guideline), do not
stop to take a detailed history – early treatment is lifesaving.

Are they
NO breathing? YES

If not breathing: Decide if reaction was

severe reaction if have 2 or
Give 5 rescue breaths
more of the below:
(via bag-valve mask)
and 100% oxygen (via 1. Life threatening problem
bag-valve mask or e.g. swelling, hoarseness,
face mask) stridor, cyanosis, signs of
shock, decreased GCS,
increased work of
If no pulse, TREAT AS A breathing
CARDIAC ARREST. 2. Sudden onset and rapid
Start CPR 15:2 and progression
follow BLS algorithm 3. Urticaria and
angioedema
If severe OR unsure, treat as
below (if no signs of severe
reaction, treat as allergic
reaction – on next

If no improvement
Give IM adrenaline (do not
 Repeat same dose of give IV) IMMEDIATELY
adrenaline every 5-15
(see Table XX for doses)
minutes.
 Involve anaesthetics and
prepare for intubation. Further treatment:
33
 Consider inotropes (see  -Give 100% oxygen.
shock guideline).  - Fluid bolus IV/IO
 Escalate to ICU/tertiary (20mls/kg RL/NS)
centre.  - Hydrocortisone IV (see
 - Nebulised salbutamol for
Table xx. Doses (improve title)
Drugs in anaphylaxis < 6 Mo 6 Mo – 6 Yr 6 – 12 Yrs >12 Yrs

Adrenaline/
epinephrine IM 300 µg 500 µg
150 µg
(0.3 ml of (0.5ml of
(Repeat every 5 -15 (0.15mls of 1:1000)
1:1000) 1:1000)
mins as needed)

Hydrocortisone IV 25mg 50 mg 100 mg 200 mg

Chlorphenamine 250 µg /kg

IM/IV (max 4 max 2.5 mg 5 mg 10 mg
doses/day) 2.5mg

Mild and mode rate allergic reactions

Not all allergic reactions cause anaphylaxis.
Symptoms and signs of mild and moderate allergic reactions are
listed below:

Mild allergic reaction Moderate allergic reaction

Mouth Itching, nausea, As mild; plus red throat, cough, mild wheeze,
urticaria, conjunctivitis diarrhoea, sweating, tachycardia, pallor.
If any life-threatening signs:- treat as
anaphylaxis urgently.

Management
 Remove the precipitating allergen if able
 Oral chlorphenamine for 24 hours.
o < 2 years 1 mg BD
o 2-5 years 1mg QID
o 6-11 years 2mg QID
o 12+ years 4mg QID
 Oral prednisolone for 3 days.
o 1 mg/kg (max dose 40 mg) OD
 Observe for at least 4 hours after treatment before discharge
home.

34
 Safety net advice for parents – warn regarding rebound
allergic symptoms and urgent medical attention if signs of
anaphylaxis.

Refere nces
APLS 6e. Advanced Life Support Group 2017.

Pocket Book of Hospital Care for Children. Second Edition.

Geneva. World Health Organisation 2013
Joint Formulary Committee. British National Formulary for
Children (Online) London. BMJ Group and Pharmaceutical
Press 2019.

35
ASPIRIN
Aspirin is used as an analgesic, an antipyretic and an anti-
inflammatory agent.

Clinical signs
 Tinnitus, rapid breathing, vomiting, dehydration, fever and
double vision are early signs.
 Later signs include drowsiness, confusion, bizarre behaviour,
unsteady walking and coma.
 Respiratory alkalosis and metabolic acidosis
 Rhabdomyolysis (dark urine), acute renal failure and
respiratory failure may occur.

Management
 Plasma salicylate concentration should be measured in all
patients with suspected toxicity where available.
 The acute toxic dose of salicylates is generally considered to
be > 150 mg/kg
 Gastrointestinal decontamination: Activated charcoal, 1 g/kg
(max: 50 g) within four hours of potential toxic ingestion
 Give IV sodium bicarbonate 8.4 % 1 mmol/kg over 4 hours to
correct acidosis and raise urine pH to above 7.5
 Give maintenance IV fluids unless the child shows signs of
dehydration
 Give supplemental potassium: 20-40 mEq/L to the
maintenance fluids used
 Monitor blood glucose every 6 hours
 Where plasma salicylate concentration is available then
treatment can be given according to Table xxx below:

36
Table xxx (Title)
Poisoning Mild Moderate Severe
Plasma salicylate < 350 mg/l > 350 mg/l > 700 mg/l

Fluids Encourage Intravenous fluids Intravenous

oral fluids [two thirds fluids [two thirds
normal normal
requirement] requirement]

Sodium No Yes Yes

bicarbonate 1
mEq/kg IV bolus
then a continuous
infusion of 100 -150
mEq in 1 L of 5%
Dextrose to run at 2
times maintenance
Consider repeated No Yes Yes
doses of activated
charcoal
Urgent referral for No No Yes
haemodialysis

Refere nces
Kliegman R, Stanton B et al; Nelson Textbook of Paediatrics
20th Edition, Elsevier, 2016

Pocket book of Hospital Care for Children: Guidelines for the

Management of Common Illnesses With Limited Resources
WHO 2005

Vree TB, Van Ewijk-Beneken Kolmer EW, Verwey-Van Wissen

CP, Hekster YA. Effect of urinary pH on the pharmacokinetics
of salicylic acid, with its glycine and glucuronide conjugates
in human. Int J Clin Pharmacol Ther 1994; 32:550.

37
ORGANOPHOSPHATE POISONING

Source
Insecticides, rat poison, etc.

Clinical manifest atio ns

Suspect in a patient with miosis, excessive salivation.

Muscarinic signs and sympto ms:

Diaphoresis, emesis, urinary and faecal incontinence, excessive
lacrimation, drooling, bronchorrhoea and bronchospasm, miosis,
hypotension and bradycardia

Nicotinic signs and sy mptoms:

Muscle weakness, fasciculations, tremors, hypoventilation,
hypertension, tachycardia, and dysrythmias

CNS effects
Malaise, confusion, delirium, seizures and coma.

Management:

Decontaminate (wash skin with soap and water, remove all

clothing if necessary)

Clear and maintain airway, give oxygen if SpO2 < 92%

Intravenous fluid and electrolyte support

Atropine 0.02 - 0.05 mg/kg (max dose = 2 mg) every 10 – 20

min.

 Give atropine until heart rate and blood pressure are normal
for age, chest is clear, sweating stops and pupils are dilated
(Full atropinisation).
 Pralidoxime [in combination with atropine] at 20-50
mg/kg/dose. Repeat in 1-2 hours if muscle weakness has not
been relieved, then 10 to 12 hours intervals if cholinergic signs
recur.
38
 For management of seizures, (refer to seizure management).

HYDROCARBONS POISONING
Examples
Petrol, Kerosene, Lighter Fluid, Paraffin Oil, Diesel Fuel, Lubricating
Oil, Furniture Polishes, Essential oils, Mineral Turpentine

Clinical signs
Transient mild CNS depression, aspiration is characterized by
coughing. Respiratory symptoms may remain mild or may rapidly
progress to respiratory failure. Fever occurs and may persist for as
long as 10 days after aspiration.

Management
 Emesis is contraindicated because of the risk of aspiration.
 Gastric lavage is also contraindicated.
 If hydrocarbon-induced pneumonitis develops, respiratory
treatment is supportive.
 Corticosteroids should be avoided, because they are not
effective and may be harmful
 Do CXR after 24 hrs if signs of respiratory distress

IRON OVERDOSE
If Iron from overdosing of antenatal ferrous sulphate drugs
(maternal), ensure to estimate amount of tablets taken….

Clinical features:
 Nausea, vomiting, abdominal pain and diarrhoea. The vomit
and stools are often grey or black.
 In severe poisoning, there may be gastrointestinal bleeding,
hypotension, drowsiness, convulsions and metabolic acidosis.
Gastrointestinal features usually appear within the first 6 hr,
and a child who has remained asymptomatic for this time
probably does not require an antidote.

Management

39
 Consider a gastric lavage if potentially toxic amounts of iron
were taken.
 Do not give activated charcoal
 Decide whether to give the antidote. As this can have side-
effects, it should
 be given only if there is clinical evidence of poisoning (see
above).
 Start fluid boluses of 20ml/kg of normal saline or ringer’s
lactate as children with iron overload are hypotensive.
 Do a chest x-ray in case of iron tablets as they may be seen
 If clinical signs are present give deferoxamine, preferably by
slow IV infusion: initially 15 mg/kg/hour, reduced after 4–6 h so
that the total dose does not exceed 80 mg/kg in
 24 h. Maximum dose, 6 g/day.
 If deferoxamine is given IM: 50 mg/kg every 6 h. Maximum
dose, 6 g/day.
 Stop infusion if patient is clinically stable. This is usually within 24
hours

Refere nces
Fiona Jepsen, Mary Ryan. Poisoning in children; Current
Paediatrics, 2005; 15: 563-568.

Carol K taketomo, Jane H Hodding, Donna M Kraus.

Paediatric Dosage Handbook.

Kliegman, Berhman, Jenson and Stanton. Nelson Textbook of

Pediatrics, 18th edition.

World Health Organization. (2013). Pocket book of hospital

care for children: guidelines for the management of
common childhood illnesses, 2nd ed. World Health
Organization.

40
PARAQUAT POISONING
Paraquat (1,1′-dimethyl-4,4′-dipyridylium) is a broad-spectrum
liquid herbicide associated with both accidental and
nonaccidental ingestion, leading to severe and often fatal
toxicity. It is rapidly but incompletely absorbed and then largely
eliminated unchanged in urine within 12–24 h.

Clinical Features
Clinical features are largely due to intracellular effects. Paraquat
generates reactive oxygen species which cause cellular
damage via lipid peroxidation, mitochondrial damage and
apoptosis in many organs:
 Vomiting,
 Fever,
 Tachycardia
 Tachypnea,
 Occasional diarrhea.
 Drowsiness
 Paraquat is actively taken up against a concentration
gradient into lung tissue leading to pneumonitis and lung
fibrosis.
 It also causes pancreatic, renal and liver injury.

Investigations
 Electrolytes, renal and liver function tests, full blood count,
should be done at least daily.
 A chest radiograph (if pneumomediastinum, pneumothorax or
lung fibrosis is suspected).
 A CT scan of the chest (useful in detecting early lung fibrosis or
assessing long-term damage in survivors).
 Amylase and lipase (acute pancreatitis suspected if patients
develop abdominal pain and a raised blood sugar).
 Plasma paraquat concentrations
 Urine and plasma dithionite tests

41
Management
 Gastric lavage is helpful.
 Activated charcoal (1gm/kg of activated charcoal by gastric
tube every 2 hours 3 to 4 doses) routinely given to minimize
further absorption.
 Supplemental oxygen should be withheld because oxygen
may contribute to the pulmonary damage, mediated through
lipid peroxidation.

Refere nces
Buckley NA, Karalliedde L, Dawson A, Senanayake N,
Eddleston M. Where is the evidence for treatments used in
pesticide poisoning? Is clinical toxicology fiddling while the
developing world burns? J Toxicol Clin Toxicol 2004;4: 113–6.

Manuel C, Gunnell DJ, Van der Hoek W, Dawson A,

Wijeratne IK,Konradsen F. Self-poisoning in rural Sri Lanka:
small-area variations in incidence. BMC Public Health 2008;
8:26.

Dawson AH, Eddleston M, Senarathna L, Mohamed F,

Gawarammana I, Bowe SJ, Manuweera G, Buckley NA.
Acute human lethal toxicity of agricultural pesticides: a
prospective cohort study. PLoS Med 2010;7:e1000357.

Bronstein AC, Spyker DA, Cantilena LR Jr, Green JL, Rumack

BH, Giffin SL.2008 annual report of the American Association
of Poison Control Centers’ National Poison Data System
(NPDS):26th annual report. ClinToxicol (Phila) 2009;47:911–
1084.

Bus JS, Aust SD, Gibson JE. Lipid peroxidation: a possible

mechanism for paraquat toxicity. Res Commun Chem
Pathol Pharmacol 1975;11:31–8.

42
DIABETIC KETOACIDOSIS (DKA)

Definition
Diabetic ketoacidosis (DKA) is the state of uncontrolled
catabolism associated with insulin deficiency, resulting in
hyperglycaemia (RBS >11mmol/L), osmotic diuresis, and
dehydration and ketonaemia (>3mmol/L) Acidaemia (blood pH
<7.3), significant ketonuria (2+or more or standard urinalysis). It is
the commonest endocrine emergency usually encountered in
previously diagnosed diabetics.

Causes
It is caused by increased fatty acid metabolism and the
accumulation of fatty acids due to reduced insulin activity or
deficiency of insulin, an intercurrent illness or it could just be a
new presentation.

Clinical features
 Dehydration
 Abdominal pain
 Ketone smell in the breath
 Acidosis
 Acidotic breathing/hyperventilation
 Unexplained coma
 Nausea
 Vomiting

In DKA, a child may die from hypokalaemia or cerebral

oedema. Cerebral oedema is unpredictable, occurs more
frequently in younger children and new diabetics, and has
mortality of around 80%.
These guidelines are intended for the management of children
who are more than 5% dehydrated and/or vomiting, drowsy or
clinically acidotic.

43
Investigations
 Random glucose
 U/E, Creatinine, LFTs
 FBC
 Malaria parasite
 Urinalysis
 Urine m/c/s
 Arterial blood gases

Treatme nt
General resuscitation: A, B, C.

Airway: Ensure that airway is patent. If comatose, insert an

airway. If comatose and has recently vomited, insert a NGT,
aspirate and leave on open drainage.

Breathing: Give 100% oxygen where indicated. Bag and mask

ventilation if apnoeic.

Circulation: Insert two large bore IV cannulae and take blood

samples.

If shocked (Tachycardia with poor capillary refill time or

hypotension) give 20 ml/kg 0.9 saline as quickly as possible, and
repeat if necessary up to a max of 60mls/kg.

Confirm the diagnosis:

 History: Polydypsia, polyuria
 Clinical: Acidotic respiration; dehydration; drowsiness;
abdominal pain/vomiting
 Biochemical: High glucose, ketones or glycosuria, ketonuria
 Conscious Level
o GCS or BCS
o Cerebral oedema: Irritability, slow pulse, high BP. Fundal
examination.
44
 Look for the focus of infection and treat accordingly.

 Observation to be carried out:

o Strict fluid balance chart
o Hourly blood glucose
o Daily weights
o GCS or BCS
o ECG

Fluids
Requirement = maintenance + deficit

Deficit = % dehydration x body weight

Maintenance= 100mls/kg-1st 10kgs, 50mls/kg- next 10kgs,
20mls/kg for every kg thereafter.
Add deficit and maintenance and give over the next 36 – 48 hrs

Example:
For a 25kg diabetic patient, fluid is calculated as follows:
Deficit= 10% x 25 = 2.5L =2500mls, to be given over 48 hrs,
therefore 1250mls to be given over 24hrs

Maintenance = 1000 mls + 500 mls +100mls = 1600mls/24hrs

Therefore, total amount of fluid to be given over 24hrs is
1600mls+1250mls =2850mls
This may be divided by 3 or 4 to be given at 950 mls 8 hourly or
~710 mls 6 hourly respectively.
Type of fluid is 0.9% normal saline. Change to DNS once RBS has
fallen to around 12 mmol/l.
In the presence of hypernatraemia, then half normal saline must
be used (please consult).

Potassium

45
Commence potassium immediately unless anuria is suspected or
there are peaked T waves on ECG or the serum potassium is > 7
mmol/l.
Add 10 to 20 mmol KCl to every 500 ml intravenous fluids given
for the first 24 hrs.

Insulin
Soluble insulin e.g. 0.1 IU/kg/hour continuous intravenous infusion.
Average rate of fall of blood glucose must not exceed 5mmol/l
per hour. If the drop in blood sugar is more rapid (>5mmol/l per
hour), dose may be reduced to 0.05 IU/Kg/hour to avoid
cerebral oedema.

Refere nces
David Southall International Child HealthCare. A practical
Manual for Hospitals Worldwide. First Edition, Book power
2003. Pages 213- 216. UK.

Richard E. Behrman, Nelson Textbook Of Paediatrics, 17 th

Edition, 2004. Pages 1954 – 1959. USA.

Sharma S, Kochar GS, Sankhyan N, Gulati S. Approach to the

Child with Coma.

Department of Pediatrics, Child Neurology Division, All India

Institute of Medical Sciences, New Delhi, 110029, India.
Indian J Pediatric. 2010 Sep 10. [Epub ahead of print]

46
ADRENAL CRISIS
Definition
Severe adrenocortical insufficiency resulting in peripheral
shutdown, cyanosis, tachycardia, tachypnoea, hypotension,
drowsiness and coma. It can be fatal if not quickly recognised
and urgently treated.

Causes
It may be due to several processes e.g.
 Congenital
o Congenital adrenal hyperplasia
o Congenital adrenal hypoplasia
o Adrenoleukodystrophy
 Acquired
o Acute haemorrhagic destruction of both adrenal glands
in previously well children due to sepsis especially
meningococcemia
o Rapid withdrawal of steroids
o Autoimmune process (Addison’s disease)
o Tuberculosis
o Birth Asphyxia
o Hypopituitarism

Clinical Features
 Vomiting/diarrhoea
 Weight loss
 Nausea

47
 Dehydration
 Hypotension
 Signs of shock
 Acidosis
 Convulsions
 Meningococcaemia-high fever, rash (petechiae,
ecchymoses, purpura, dermal gangrene, neck stiffness
 TB-weight loss, fever, cough,, night sweats, reduced appetite
with positive history of TB contact

Investigations
 Random glucose (hypoglycaemia)
 Urinalysis (ketosis)
 ECG (Hyperkalaemia)
 Serum Cortisol - <3µg/dL in the morning (09.00) defines low
cortisol
 ACTH- often high
 U/Es (Hyponatraemia, Hyperkalaemia), Creatinine – (GFR
decreases)
 Aldosterone - levels are often normal
 Urinary Na and Cl increased, K is decreased
 Abdominal U/S, CT scan, MRI - size of adrenal glands
 CXR- miliary picture or opacities suggestive of TB
 Gene Xpert

Treatme nt
 Airway
 Breathing
 Circulation – correct shock with normal saline at 20 mls/kg,
then correct dehydration
 Treat hypoglycaemia
 Normal saline to correct salt deficit.
 Hydrocortisone 8mg/kg start then
48
 Hydrocortisone 4mg/kg 6 hourly daily maintenance
 Treat underlying cause
Note: Dexamethasone has no mineralocorticoid activity and so
should not be used to treat adrenal crisis

Refere nces

David Southall, International Child Health Care, A practical

Manual for Hospitals worldwide. First edition, Book Power,
Page 219. 2003, UK.

Behrman, Nelson Textbook of Paediatrics, 17th Edition, Page

1904, Saunders 2004. USA.

PJ Simm, CM McDonnell and MR Zacharin.16 th April 2004

Department of Endocrinology and Diabetes, Royal

Children’s Hospital, Melbourne, Victoria, Australia .Review of
Primary Adrenal insufficiency In Childhood And
Adolescence. Advances In Diagnosis and Management.

49
CARDIOVASCULAR SYSTEM

CONGESTIVE CARDIAC FAILURE (CCF)

Definition
CCF is a clinical syndrome in which the heart:
 Is unable to pump enough blood to the body to meet its
needs,
 Is unable to dispose of venous return adequately,
 OR a combination of the two.
Diagnosis of CCF relies mainly on clinical findings and no single
test is specific.

Aetiolo gy
CCF may result from congenital or acquired heart diseases with
volume and/ pressure overload or from myocardial insufficiency.

Table XX
Cause Example/s
1. Cardiac
A. Congenital
 Ventricular Septal Defect (VSD)
 Atrial Septal Defect (ASD)
 Patent Ductus Arteriosus (PDA)
 Arrhythmias
B. Acquired
 Endocardial/Valvular disease e.g.
Rheumatic Heart Disease (RHD)
 Myocardial diseases e.g. Viral
myocarditis, Dilated
cardiomyopathies, hypertrophic
cardiomyopathy
 Pericardial diseases e.g. TB pericarditis

2. Extracardiac

50
  Anaemia
 Pulmonary diseases e.g. Pulmonary hypertension,
 Severe pneumonia especially in neonate
 Systemic hypertension
 Metabolic disorders e.g. Electrolyte imbalances, hypoglycaemia
 Endocrine e.g. Thyroid disease
 Drugs e.g. antineoplastic

Diagnosis
The diagnosis is based on:
 History: Poor feeding, FTT, Difficulties in breathing, poor weight
gain, easy fatiguability in older children.
 Examination: tachypnoea, tachycardia, hepatomegaly, +/-
Cardiac murmurs, oedema in older children.
 Investigations: FBC, U&Es, Serum Creatinine,
Echocardiography, Chest x-ray and ECG.

Aims o f tre atment

 To relieve symptoms
 Elimination of the underlying/precipitating cause
 Improve survival

General meas ures

 Propped up position
 Oxygen
 Salt and fluid restriction
 Daily weight in hospitalised patients

Drug t herapy
The cornerstones of management are:
 Preload reduction: Diuretics e.g. Frusemide
 Afterload reduction: ACEIs (Captopril, Enalapril),
 Inotropes: digoxin (stable patient), Dobutamine/dopamine
(Severe CCF)
 β-blockers e.g. Carvedilol (used in chronic heart failure state
typically in dilated cardiomyopathies)

51
Dosages Table XX
Drug Route Dosage
Frusemide IV 1mg/kg/dose, 2-3 times a day
Oral
1-2mg/kg/dose, 1-3 times a day
Spironolactone Oral 1-2mg/kg/dose, 1-2 times a day
Hydrochlorothiazide Oral 2-4 mg/kg/day in 2 – 3 divided
Digoxin Oral 0.02 - 0.05 mg/kg OD PO
Captopril Oral 0.1-0.5mg/kg Divided 8hourly,
max. dose 0.6mg/Kg
Enalapril Oral 0.1mg/Kg divided OD or BD,
Max. dose 0.5mg/kg/day
Carvedilol Oral 0.08 mg/kg 12 hourly, if tolerated
increase by 0.08 mg/kg every 1-2
weeks to a maximum 0.50mg/kg
12 hourly.

NOTE: If in cardiogenic shock and requiring Dopamine,

Dobutamine or Milrinone, refer to the shock protocol.

52
ACUTE RHEUMATIC FEVER

Diagnosis
Diagnostic criteria for rheumatic fever is done according to the
modified 2015 Jones criteria. Has defined high risk population
recognizing variability in clinical presentation and had included
Echocardiography as a tool to diagnose cardiac involvement
(for subclinical carditis). (Zambia is in a high-risk category).

Modified 2015 Jones’ c riteria

Major Minor
Carditis (clinical or subclinical) Monoarthralgia
Arthritis – monoarthritis or Fever (≥ 38.0ºC)
polyarthritis
Polyarthralgia ESR ≥ 30 mm/hour and/or CRP ≥ 3.0
mg/dl
Chorea Prolonged PR interval (after
considering the differences related to
age; if there is no carditis as a major
criterion)
Erythema marginatum
Subcutaneous nodules

ESR – erythrocyte sedimentation rate; CRP – C-reactive protein

(Adapted from ref 13). All patients with ARF should have an
Echocardiography done even in the absence of clinical
suspicion of valvular done.

Revised Jones C riteria

WHO guidelines set the international standard for diagnosis of
ARF:
Two major manifestations plus evidence of preceding
streptococcal infection OR one major and two minor
manifestations plus evidence of preceding streptococcal
infection.

Management
Treat the illness

53
1. Benzathine penicillin injection stat or oral penicillin for ten
days
2. Relieve symptoms
o Bed rest
o Relief of arthritis, pain and fever
o Treat chorea (if severe)
o Anti- heart failure medication (see table 2 above)

Recommended Anti-inflammatory Agents

Arthritis Carditis
Prednisolone Nil 2 – 4 weeks

Aspirin 1 – 2 weeks Until symptoms subside

*Ibuprofen Until symptoms subside Until symptoms subside

Dosages: Prednisolone 2 mg/kg/day in four divided doses

Aspirin 50 to 60 mg/kg/day in four to six divided doses
*Ibuprofen can be given at 30 mg/kg/day in 3 divided
doses, where Aspirin not tolerated.

The dose of prednisolone should be 2 mg/kg/day (max 60 mg);

then taper by 20–25% per week. Aspirin can be reduced to 25 to
30 mg/kg/day when symptoms improve. The dose of
prednisolone should be tapered, and aspirin started during the
final week.

Management o f Syde nham’s chorea

1. Reduce physical and emotional stress and use protective
measures as indicated
2. Benzathine penicillin IM stat (Eradicate GAS), then every 28
days for secondary prophylaxis.
3. Anti-inflammatory agents not indicated
4. For severe chorea, any of the following drugs may be used:

54
 o Carbamazepine 7–20 mg/kg/day (7–10 mg/kg day
usually sufficient) given TDS PO until chorea is controlled
for at least 2 weeks, then trial off medication
o Valproic acid Usually 15–20 mg/kg/day (can increase to
30 mg/kg/day) given TDS PO until chorea is controlled
for at least 2 weeks, then trial off medication.
o Phenobarbitone, Haloperidol and Chlorpromazine can
be used when above not available.

Further manage me nt plan

 Baseline echocardiography (ECHO)
 ARF register (cardiac clinic), issue ARF prophylaxis card
 Education of patient and family
 Dental examination
 Long term secondary prophylaxis plan

Secondary pro phylaxis to prevent rec urrent ARF is a

long term, regular administ ration of antibiotics to:
 Prevent group A β-Haemolytic Streptococcal (GAS)
pharyngitis
 Prevent repeated development of ARF
 Prevent development of rheumatic heart disease (RHD)
 Reduce severity of RHD
 Help reduce the risk of death from severe RHD

Antibiotic regimens for secondary prophylaxis

(Adapted from ref 14)
Antibiotic Dose Route Frequency
First line
Benzathine 1,200,000 U (900 mg) Deep IM 4-weekly
Penicillin G ≥ 30 Kg Injection
600,000 U (450 mg)
≤30 Kg
Second line (If IM route is not possible or refused, adherence should be
carefully monitored)

55
 Phenoxymeth 250mg Oral Once daily
ylpenicillin
(Pen V)
Following documented penicillin allergy
Erythromycin 250mg Oral Twice daily

NOTE: Duration of prophylaxis in all persons with ARF is for a

minimum of 10 years after the most recent episode of ARF or until
age 21 years (whichever is longer). For RHD, the duration of
prophylaxis is for life.

56
RHEUMATIC HEART DISEASE
Rheumatic hear disease (RHD) is includes a spectrum of lesions
from pericarditis, myocarditis, and valvulitis during ARF, to
chronic valvular lesions that evolve over years following one or
more episodes of ARF.

The common valvular lesions in RH D

 Mitral regurgitation/stenosis
 Aortic regurgitation/stenosis
 Tricuspid regurgitation/stenosis

Best practice fo r RHD requires:

 Access to echocardiography (for diagnosis)
 Secondary prevention with penicillin prophylaxis (see above
on ARF)
 Access to oral healthcare
 Access to a specialist physician, paediatrician and/ or
cardiologist
 Adequate monitoring of anticoagulation therapy in patients
with atrial fibrillations (AF) and/or mechanical prosthetic
valves (where possible)
 Access to cardiothoracic and interventional cardiology
services (where possible).

RHD pat ients may present with:

 Heart failure (as a result of valvular insufficiency or stenosis)
and/or
 Complications such as atrial arrhythmias, pulmonary oedema,
recurrent pulmonary emboli, infective endocarditis,
intracardiac thrombus formation, and systemic embolism.

Treatme nt of RH D
 Medical:
o Prevent ARF (Elimination GAS pharyngitis as above)
o Supportive treatment for CCF (as above)
o Prevent recurrent ARF in children with RHD (see
secondary prophylaxis above)
57
 o Monitoring for the complications and sequelae of
chronic RHD.
 Surgery:
o Indicated in patients with persistent CCF OR
o Worsening after aggressive medical therapy for RHD to
decrease valve insufficiency/regurgitation.

58
INFECTIVE ENDOCARDITIS
Infective endocarditis (IE) is defined as an infection of the
endocardial surface of the heart (heart valves and mural
endocardium) by microorganisms (mainly bacteria) hence also
called bacterial endocarditis.

Risk factors
 Congenital heart disease especially Cyanotic CHD
 Rheumatic heart disease
 Prosthetic heart valve
 History of endocarditis
 IV drug use or chronic IV access
 Immunocompromised (HIV, diabetes)

Classificatio n
1. Acute infective endocarditis
Caused by virulent organisms, like S. aureus, enterococci
and streptococcus, which are harmful even on healthy
endocardium. Onset of disease is stormy with high grade
fever and causes destructive lesions on the endocardium
like ulcerations, perforation, regurgitation and ring abscesses
especially around prosthetic valves.

2. Subacute infective endocarditis

Caused by relatively low virulent organisms e.g S.viridans
and HACEK group (Haemophilus, Aggregatibacter,
Corynebacterium, Eikanella, Kingelle). It runs a more insidious
course:
o low grade fever, anorexia, weight loss, influenza like
syndromes, myalgia, pleuritic pain
o No specific heart pathological features. Characterised
by slowly growing chronic inflammation, fibrosis and with
tightly held endocardial vegetations. Chronicity of this
type of IE causes chronic antigenemia which in turn is
prone to immune complex formation.

Diagnosis

59
A fever with new/or changing murmur is IE until proven otherwise.

Dukes criteria for the diagnosis of IE

Major criteria Minor criteria

Positive blood culture Predisposition: predisposing

heart condition or IV drug use
Typical microorganism consistent with IE
from ≥2 blood cultures, Fever: temperature ≥38.0°C

Microorganisms consistent with IE from Vascular phenomena: major

persistently positive blood cultures, arterial emboli, septic
defined as: pulmonary infarcts, mycotic
aneurysm, intracranial
≥2 Positive cultures of blood samples haemorrhage, conjunctival
drawn >12 h apart or haemorrhages, and Janeway
All of 3 or a majority of ≥4 blood cultures, lesions
irrespective of the timing Immunologic phenomena:
1 Positive blood culture for Coxiella glomerulonephritis, Osler nodes,
burnetii or antiphase-I immunoglobulin G Roth’s spots, and rheumatoid
antibody titre >1:800 factor

Evidence of endocardial involvement Microbiological evidence:

positive blood culture but does
Positive echocardiogram, defined as: not meet a major criterion as
noted above or serological
Oscillating intracardiac mass on valve or evidence of active infection
supporting structures, in the path of with organism consistent with IE
regurgitant jets, or on implanted material
in the absence of an alternative
anatomic explanation or

Abscess OR

New partial dehiscence of prosthetic

valve OR

New valvular regurgitation (worsening or

changing of pre-existing murmur not
enough)

Diagnosis is made when there are 2 major criteria or 1 major plus

3 minor or 5 minor criteria. Adapted from ref 15

60
Treatme nt
Drugs Dosage Duration Remarks
1st Line Crystalline Penicillin (X Pen) Then,
50-100,000 IU/kg in 4 divided 4 weeks Ciprofloxacin
doses/day 15mg/kg/day in 2
And, Gentamicin divided doses for 2
weeks
3-5mg/kg/day in 2-3 divided
doses/day
Ceftriaxone 80- Then,
100mg/kg/day 1-2 times 4 weeks Ciprofloxacin
daily/day 15mg/kg/day in 2
And, Gentamicin 3- divided doses for 2
5mg/kg/day 2-3 divided weeks
doses/day
2nd Line Vancomycin 30- Then,
40mg/kg/day in 4 divided 4 weeks Ciprofloxacin
doses 15mg/kg/day in 2
And, Gentamicin divided doses for 2
3-5mg/kg/day in 2-3 divided weeks
doses

61
SYSTEMIC HYPERTENSION

Definitions
 Hypertension: Systolic and/or diastolic pressure levels greater
than the 95th percentile for age and gender on at least three
occasions. BP readings of 5mmHg or more above the 99th
centile values are considered as severe hypertension.
 Pre-hypertension: average systolic or diastolic blood pressure
between the 90th and 95th percentiles for age and gender.

To insert table for normal values

Aetiolo gy ///Are we going to stic k with caus e or

aetiology ?/
 Essential or primary hypertension, in which a specific aetiology
cannot be identified
 Secondary hypertension in which a cause can be identified
(commonest in paediatric population)
More than 90% of the cases of secondary hypertension are
caused by chronic renal disease, renovascular disease and
coarctation of the aorta.

Commo nest c auses of secondary hypertensio n in

childre n
Examples of lesions/pathology
Renal Acute and chronic Glomerulonephritis
Acute and chronic Pyelonephritis
Congenital anomalies [polycystic or dysplastic
kidneys]
Obstructive uropathies [hydronephrosis]
Haemolytic-uremic syndrome
Renal damage from nephrotoxic medications,
trauma or radiation
Renovascular Renal artery disorders (e.g. stenosis, polyarteritis,
thrombosis)
Renal vein thrombosis

62
 Cardiovascular Coarctation of the aorta
Conditions with large stroke volume [PDA, aortic
insufficiency, system A-V fistula, complete heart
block]. These conditions cause only systolic
hypertension

Endocrine Hyperthyroidism (systolic hypertension)

Excessive catecholamine levels
(Pheochromocytoma, Neuroblastoma)
Adrenal dysfunction
Cushing’s syndrome
Hyperaldosteronism
Neurogenic Increased intracranial pressure

Drugs and Steroids

chemicals Sympathomimetic drugs [cough medications]
Heavy metal poisoning [mercury, lead]
CNS stimulants: Cocaine [acute or chronic use]

Most commo n causes of hype rtension by age groups

Age Causes
Newborns Renal artery stenosis, renal artery thrombosis,
congenital renal malformation coarctation of the
aorta, bronchopulmonary dysplasia
< 6 years Renal parenchymal disease, Coarctation of the aorta,
Renal artery stenosis
6 -10 years Renal artery stenosis, renal parenchymal disease,
primary hypertension
>10 years Primary hypertension, renal parenchymal disease

Diagnosis and work-up

 Mild hypertension is usually asymptomatic and may only be
picked up incidentally
 Severe hypertension may be symptomatic (headache,
dizziness, nausea and vomiting, irritability, personality
changes) occasionally presenting with neurological
manifestation, CCF, renal dysfunction, and stroke.
 History, physical findings and laboratory tests usually point to
the cause of hypertension.

63
Routine and special laboratory tests and t heir
significance
Routine Laboratory Tests Significance of Abnormal Results
Urinalysis, urine culture, urea Renal parenchymal disease
Creatinine, uric acid
Serum electrolyte Hyperaldosteronism 1° or 2°, Adrenogenital
(hypokalaemia) syndrome, renin producing tumours
ECG, CXR, ECHO Cardiac causes e.g. coarctation of the aorta
Specialised tests
IVU, US KIDNEY, CT scan Renal parenchymal disease, renovascular
disease, tumours (neuroblastoma, Wilms)
Plasma renin activity High-renin hypertension
Renovascular disease
Renin-producing tumours
Some Cushing’s syndrome
Some essential hypertension
Low-renin hypertension
Adrenogenital syndrome
Primary hyperaldosteronism
24-hr urine collection for 17- Cushing’s syndrome, adrenogenital
ketosteroids and 17- syndrome
hydroxycorticosteroids
24-hr urine collection for Pheochromocytoma, neuroblastoma
catecholamine levels and
VMA
Aldosterone Hyperaldosteronism, primary or secondary
renovascular disease, renin-production
tumours
Renal vein plasma renin Unilateral renal parenchymal disease,
activity renovascular hypertension
Abdominal aortogram Renovascular hypertension, abdominal CoA,
unilateral renal parenchymal disease,
pheochromocytoma.

Management

64
As most of the causes of hypertension in children are secondary,
the ultimate aim of treatment, in addition to general measures
and pharmacological therapy should be to remove the cause of
hypertension whenever possible: e.g coarctation repair or renal
artery balloon angioplasty or surgery for renovascular disease.

No n-pharmacologic /General measures:

 Encourage weight reduction if indicated
 low salt foods/diet
 avoidance of smoking
 Regular exercise and restriction of sedentary lifestyle

Pharmacologic measures:
Whom to treat
 Symptomatic hypertension and severe hypertension
 Prehypertension in presence of comorbid conditions, such as
chronic kidney disease or diabetes mellitus
 Hypertensive children with diabetes mellitus or other risk CVD
factors, such as dyslipidaemia
 Hypertensive target-organ damage, most often left ventricular
hypertrophy (LVH)
 For essential hypertension (without any evidence of target-
organ damage) that persists despite a trial of four to six
months of nonpharmacologic therapy.

Antihypertensive drugs
Secondary hypertension is the commonest cause of
hypertension in children and the treatment of the underlying
medical conditions influences the choice of specific class of
antihypertensive drugs.
 In children with chronic kidney disease, we suggest that ACE
inhibitors be used as the initial antihypertensive agent. In
patients who cannot tolerate ACE inhibitors, angiotensin-
receptor blockers (ARBs) are a reasonable alternative.
 In adolescents with primary HTN without target-organ
damage, we suggest that low-dose thiazide diuretic therapy
be used as the first antihypertensive agent

65
 In children with either type 1 or type 2 diabetes mellitus, we
suggest that ACE inhibitors be used as the initial
antihypertensive agent. In patients who cannot tolerate ACE
inhibitors, angiotensin-receptor blockers (ARBs) are a
reasonable alternative.
 Diuretics are the cornerstone of antihypertensive drug therapy
in essential hypertension and are not used in patients with
renal failure. Their action is related to a decrease in
extracellular and plasma volume.
 ACE inhibitors are contraindicated in obstructive lesions like
bilateral renal artery stenosis, aortic stenosis and coarctation
of the aorta.

The stepped-care approach, using three clas ses of

drugs: diuretics, B-blo ckers, and vasodilators, is
popular.
Step 1

Initiate with a small dose of a single antihypertensive drug

(thiazide diuretic or adrenergic inhibitor), adjust upwards as
necessary
Step 2

If the first drug is not effective, a second drug may be added to,
or substituted for, the first drug, starting with a small dose and
proceeding to full dose.
Step 3

If the blood pressure remains elevated, a third drug, such as a

vasodilator, may be added to the regimen. At this point, the
possibility of secondary hypertension should be reconsidered.

Recommended oral dosage of selected

antihyperte nsive drugs for children
Drugs Dose Times/day
Calcium channel blockers (CCB)
 Nifedipine  0.1 -1 mg/kg/day  1-2 times a day
 Amlodipine (≥ 6 yrs old)  2.5-5mg/day  Once a day
β-Blockers

66
  Propranolol  1-3mg/kg/day  2-3 times per daily
 Atenolol  1-2mg/kg/day  Once daily
ACE inhibitors
 Captopril  0.3-0.5mg/kg/dose  3 times per day
(max 6mg/Kg/day)
 Enalapril  0.08-0.6mg/kg/day  1-2 times per day
 Lisinopril  0.07-0.6mg/kg/day  Once daily
ARB
 Losartan  0.7-1.4mg/kg/day  Once daily

Diuretics
 Hydrochlorothiazide  1-2mg/kg/day  Once daily
 Furosemide [lasix]  0.5- 2mg/kg/dose  1-2 times per day
 Spironolactone  1-3mg/kg/day  1-2 times per day
[aldactone]

Hyperte nsive c risis

A hypertensive emergency is defined as any of the following
features:
 The patient has severe hypertension [>180 mm Hg systolic or
110 mm Hg diastolic] or rapidly increasing blood pressure.
 The patient has neurologic signs [hypertensive
encephalopathy] with severe headache, vomiting, irritability,
apathy, papilloedema, retinal haemorrhage, or exudates.
 The patient has CHF or pulmonary oedema.

Aggressive administration of antihypertensive drugs is indicated to

lower blood pressure – no more than 25% reduction of systolic BP
in the first 8hours; gradually attain normal BP over 24-48 hours:

Drug Dose Comment

Nifedipine 0.2 - 0.5 mg/kg, every 4 to 6 hours, If patient is conscious
12 to 24 hourly if retard Nifedipine
is used
*maximum 10mg per dose
Hydralazine 0.15 mg/kg IV slow infusion over The dose may be
20 minutes repeated at 4 to 6
hours interval.

67
 The dose may be
repeated at 4-6 to 6-
hours interval.
Nitroprusside 1-3µg/kg per min as IV drip
Labetalol 0.2-2mg/kg/hour IV drip Alpha and beta
blocker
Diazoxide 3-5mg/kg as IV bolus
Furosemide 1mg/kg IV bolus To initiate diuresis
(*Fluid balance must be controlled carefully, so intake is limited to urine
output plus insensible loss. Seizures may be treated with slow intravenous
infusion of diazepam [Valium], 0.2 mg/kg or another anticonvulsant
medication. When a hypertensive crisis is under control, oral medications
replace the parenteral medications.)

68
CYANOTIC SPELL

Definition
Also called hypoxic spell or “tet” spell occurs in young infants
with Tetralogy of Fallot (TOF). It consists of hyperpnoea (i.e. rapid
and deep respiration) worsening cyanosis and the
disappearance of the heart murmur.
This occasionally, if not treated, results in complication of the
central nervous system and even death.

Place the infant who has a

hypercyanotic spell in the
knee-chest position. This
position increases systemic
vascular resistance in the
lower extremities.

Adapted from Child Health

Nursing: Partnering with
Children and Families, 2006.

69
Step by ste p appro ach to a hype rcyanotic spell

There’s an increase in the R-L  Keep child calm with

shunt that leads to: parents
 Hyperapnoea  Knee-chest position
 Worsening cyanosis  Oxygen if it doesn’t
cause distress
 Irritability
 Continuous oxygen
 Disappearance of murmur
saturation monitoring
 Limpness, convulsions,
death

If cyanosis persists

Morphine 0.2 - 0.5 mg/Kg PO OR buccal Midazolam 0.3 mg/Kg

Cyanosis persists?
YES

NO Site IV for NS bolus at 20mls/kg

Propranolol 1mg/kg/dose 4 hrly

Morphine can now be given IV if

available at 0.1mg/Kg

Calm Child If ABG’s available and severe

Propranolol 0.5-1mg/Kg/dose acidosis, correct with IV bicarb

If condition is worsening, consider

Ketamine 1-3mg/kg, intubation
Indicators of improved
and ventilation
PBF:
 Reduced cyanosis Correct any underlying cause:
 Murmur becomes Hypothermia, Hypoglycaemia,
louder dehydration, anaemia

70
Treatme nt
 Using the knee-chest position and holding the baby traps
systemic venous blood in the leg thereby decreasing the
systemic venous return and helping to calm the baby. The
knee-chest position may also increase SVR by reducing
arterial blood flow through the femoral arteries.
 Morphine sulphate, at 0.2 mg/kg administered SC, IM or IV
suppresses the respiratory centre and abolishes hyperpnoea.
 Sodium bicarbonate (NaHCO3) at 1 mmol/l IV. Corrects
acidosis and eliminates the respiratory centre–stimulating
effect of acidosis. The same dose can be repeated in 10 to 15
minutes
 Administration of oxygen may improve arterial oxygen
saturation a little.
 Vasoconstrictors such as phenylephrine at 0.02 mg/kg IV raise
SVR.
 Ketamine at 1 to 3 mg/kg given over 1 minute is a good drug
to use since it simultaneously increases the SVR and sedates
the patient. Both effects are known to terminate the spell.
 Propranolol at 0.01 to 0.25 mg IV slowly has been used
successfully in some cases both acute or chronic. Its
mechanism of action is not entirely clear. When administered
for acute cases propranolol may reduce the spam of the RV
outflow tract and slow the heart rate. The successful use of
propranolol in the prevention of hypoxic spell is more likely the
result of the drugs peripheral action. It may stabilize vascular
reactivity of the systemic arteries thereby preventing a sudden
decrease in the SVR.
 Oral propranolol therapy at 2 to 6 mg/kg/day in three to four
divided doses may be used to prevent recurrence of hypoxic
spell and delay corrective surgical procedures in high risk
patients.

71
 o Physicians should recognize and treat hypoxic spells. It is
important to educate parents to recognize the spell and
what to do.
o Maintenance of good dental hygiene and antibiotic
prophylaxis against SBE are important
o Relative iron-deficiency anemia should be detected
and treated. Anaemic children are more susceptible to
CVA. Normal Hb or HCT or decreased RBC indices
indicate iron- deficiency anaemia in cyanotic patients.

Refere nces:
1. Guest lecture by Dr. John starling Red Cross Children’s
Hospital
2. Park’s Paediatric Cardiology for Practitioners by Myung K
Park, 6th edition
3. World Heart Federation training manual for RF/RHD
4. Management of polycythaemia in adults with congenital
cyanotic heart disease. S A Thorne; Heart 1998; 79; 315-316
5. Cardiology by Neil R Grubb David E Newby; second edition
6. 6. Essence of Paediatrics; Third edition

7. Standard treatment guidelines, essential medicines list and

laboratory supplies list for Zambia. 1st edition 2004.
8. Advanced paediatric life support the practical approach
4th edition.
9. Drugs doses by Frank Shann; Intensive Care Uinit; Royal
children’s hospital Parkville, Victoria 3052; Australia, 13th
edition 2005.
10. Echo made easy; Sam Kaddoura
11. Clinical wizard, pocket clinical reference Guide; Mennen
medical
12. Nelson test book of paediatrics 16th edidtion
13. Gewitz M, Baltimore R, Tani L, et al. Revision of the Jones
Criteria for the Diagnosis of Acute Rheumatic Fever in the Era
72
 of Doppler Echocardiography: A Scientific Statement From
the American Heart Association. Circulation. 2015;131:1806–
1818. [PubMed] [Google Scholar]
14. RHD Australia (ARF/RHD writing group), National Heart
Foundation of Australia and the Cardiac Society of Australia
and New Zealand. Australian guideline for prevention,
diagnosis and management of acute rheumatic fever and
rheumatic heart disease (2nd edition). 2012. Available at:
https://www.rhdaustralia.org.au/system/files/fileuploads/rhd
_prevention_diagnosis_management.pdf
15. Baltimore R S, Gewitz M, Baddour M et al., 2015. Infective
Endocarditis in Childhood: 2015 Update A Scientific
Statement from the American Heart Association. American
Heart Association. Available at:
https://ahajournals.org/doi/pdf/10.1161/CIR.00000000000002
98

73
THE RESPIRATORY SYSTEM

CHILD WITH STRIDOR

Definition
Stridor is a harsh noise during inspiration, which is due to narrowing
of the airway from the oropharynx, glottis and the trachea. It
usually occurs in inspiration but may occur in expiration.

The commonest causes of stridor in children are viral croup,

foreign body, epiglottitis and congenital anomalies especially in
neonates. Others include anaphylaxis burns and retropharyngeal
abscesses. A good history helps to establish the possible cause.

VIRAL CROUP (LARYNGOTRACHEOBRONCHITIS)

Introduction
 Croup (laryngotracheobronchitis) is a common cause of
upper airway obstruction in toddlers (1-3 years old)
 It is characterised by varying degrees of inspiratory stridor,
barking cough, and hoarseness due to inflammation in the
laryngeal region

Clinical Presentation
 Low grade fever and coryzal symptoms are followed over 12–
24 h by a harsh, barking cough
 Stridor is most evident when the child is upset or agitated
 Respiratory distress of varying degrees
 Usually, resolves spontaneously over a 3-4 day period

Diagnosis
 Croup is a clinical diagnosis
 Neck x-rays are unnecessary unless the diagnosis is in doubt
 Important differential diagnoses
o Acute epiglottitis
o Bacterial tracheitis
o Foreign body inhalation

74
 o Anaphylaxis
o Retropharyngeal abscess
Table XXX. Assessment of severity
Sign Mild croup Moderate croup Severe croup
Stridor Only when At rest Severe, biphasic
agitated
Recession Mild subcostal Moderate Use of accessory
tracheal tug muscles
Level of Restless when Anxious, agitated Lethargic, drowsy
consciousness disturbed

Management
 Keep the child on the mother’s lap and handle gently
 Do not attempt to forcefully examine the oropharynx
 See flow chart below:

75
 Assess level of
severity

Mild croup Moderate croup Severe croup

Dexamethasone Dexamethasone 0.6 Dexamethasone 0.6

mg/kg PO stat (max mg/kg PO stat (max
0.6 mg/kg PO stat
12 mg) 12 mg)
(max 12 mg)
Or prednisolone 2 Start nebulised
Or prednisolone 2
mg/kg PO stat adrenaline
mg/kg PO stat
immediately
Nebulised
Or nebulised
adrenaline 1:1000 Give oxygen 1-3
budesonide 2 mg
(1-5mls) diluted in litres/min
stat
0.9% normal saline
Admit ICU/HDU or
Observe for 4
Can be repeated refer to centre
hours
every 30 min (2 to 3 where intubation
Discharge after 4 times) /tracheostomy can
hours if child be done.
*Admit acute area
stable and well
improving

* As airway obstruction can occur suddenly, ensure that facilities for an

emergency intubation and/or tracheostomy are immediately available if
required.

Ca ut ion

 Avoid using oxygen unless there is impending airway obstruction.

Nasal prongs or a nasal or nasopharyngeal catheter can upset the
child and precipitate obstruction of the airway.
 Do not use antibiotics or bronchodilators unless diagnosis is in doubt
 Do not give adrenaline nebulisations if baseline heart rate >200/min

Refere nces

76
Cherry J.A. Croup. NEMJ 2008, January, 358; 4: 385-391

Fitzgerald D.A & Kilham H.A. Croup: Assessment and

Evidence-based Management. MJA 2003; 179: 372-377

Malhotra M & Krilov L.R. Viral croup. Pediatric Reviews. 2001;

22: 5-12

Forfar and Aneil. Textbook of Paediatrics. 6th edition

77
ACUTE EPIGLOTTITIS
Epiglottitis is inflammation of the epiglottis. The epiglottis closes
the airway during swallowing. Epiglottitis is a medical emergency
that may result in death if not treated quickly due to rapid
airway obstruction.
The incidence of epiglottitis due to Haemophilus influenza has
reduced due to Hib vaccine in the Extended program on
immunisation.

Signs and Sympto ms

 sore throat with difficulty in speaking
 difficulty in breathing
 stridor
 fever
 drooling of saliva difficulty in swallowing or inability to drink.

Management
Treatment of patients with epiglottitis is directed to relieving the
airway obstruction and eradicating the infectious agent.
 Keep the child calm in a seated or leaning forward position,
and provide humidified blow-by oxygen, with close
monitoring. Avoid laying the child in supine position.
 Avoid examining the throat if the signs are typical, to avoid
precipitating obstruction.
 Elective intubation is the best treatment if there is severe
obstruction but may be very difficult due to severe swelling;
consider the need for surgical intervention to ensure airway
patency.
 Give IV antibiotics when the airway is safe: ceftriaxone at 80
mg/kg once daily for 5 days.
 Give Paracetamol 15mg/kg qid or Ibuprofen 10mg/kg tds
when airway is secure.

78
FOREIGN BODY INHALATION
Children are usually at high risk due to the tendency of inhaling
small objects. The foreign body usually lodges in a bronchus
(more often in the right) and can cause collapse or cause
consolidation of the portion of lung distal to the site of blockage.
Choking is a frequent initial symptom. Objects such as fish bones
can lodge in the larynx, causing stridor or wheeze. Sometimes
the chocking may be followed by a symptom-free interval of
days or weeks before the child presents with persistent wheeze,
chronic cough or pneumonia, which fails to respond to
treatment.
A large object lodged in the larynx can cause death from
asphyxia, unless it can be dislodged, or an emergency
tracheostomy be done.

Diagnosis
Inhalation of a foreign body should be considered in a child with
the following:
 sudden onset of choking, coughing or wheezing; or
 segmental or lobar pneumonia that fails to respond to
antibiotic therapy.
 Examine the child for:
o unilateral wheeze
o an area of decreased breath sounds that is either dull or
hyper-resonant on percussion
o deviation of the trachea or apex beat.
 Obtain a chest X-ray at full expiration to detect an area of
hyperinflation or collapse, mediastinal shift (away from the
affected side) or a foreign body if it is radiopaque.

Treatme nt
Emergency first aid for the choking child: Attempt to dislodge
and expel the foreign body. The management depends on the
age of the child.

79
For infants:
a) Lay the infant in a head-down position
on one of your arms or on your thigh.
b) Strike the middle of the infant’s back five
times with the heel of your hand.
c) If the obstruction persists, turn the infant
over and give five firm chest thrusts with
two fingers on the lower half of the
sternum.
d) If the obstruction persists, check the
infant’s mouth for any obstruction that
Adapted from WHO
can be removed
pocketbook of care
e) If necessary, repeat this sequence with for children in hospital,
2nd ed, 2013
back slaps.

For older childre n:

While the child is sitting, kneeling or lying, strike
the child’s back five times with the heel of the
hand.
If the obstruction persists,
go behind the child and
pass your arms around the
child’s body; form a first
with one hand immediately below the
sternum; place the other hand over the fist
and thrust sharply upwards into the
abdomen. Repeat this up to five times.
 Then check the child’s mouth for any Heimlich manoeuvre
obstruction that can be removed. for a chocking older
child
 If necessary, repeat the sequence with
back slaps.
Once this has been done, it is important to check the patency of
the airway by:
80
 looking for chest movements
 listening for breath sounds and
 feeling for breath.

If further management of the airways is required after the

obstruction is removed, keep the child’s airways open and
prevent the tongue from falling back to obstruct the pharynx
while the child recovers.

Later tr eatment of suspe cted foreign body aspiration

 If a foreign body is suspected but cannot be confirmed, refer
the child to a hospital where diagnosis is possible, and the
object can be removed after bronchoscopy.
 If there is evidence of pneumonia, begin treatment with
ampicillin (or benzylpenicillin) and gentamicin, as for severe
pneumonia (see above), before attempting to remove the
foreign body.

Refere nces
Escobar ML, Needleman J. Stridor. Pediatrics in Review.
2015;36(3):135-7.

Garcia S, Ciriaci C, Montes G, Corbaz S. [Epiglottitis due to

Haemophilus influenzae type b in the vaccination era:
pediatric clinical case]. Archivos argentinos de pediatria.
2019;117(4): e403-e5.

World Health Organization. (2013). Pocket book of hospital

care for children: guidelines for the management of
common childhood illnesses, 2nd ed. World Health
Organization

81
THE WHEEZING CHILD

Introduction
Wheezing is a common presentation in young children. Diagnosis
and treatment of these children can be challenging, as arriving
at a final diagnosis often requires a process of exclusion
Without a clear diagnosis, a correct treatment approach is not
possible, and a diagnosis may become more certain depending
on the treatment response.

The algorithm shown in Fig xxx below considers the most

common primary presentations with wheeze and gives one
possible approach to this problem. A brief summary of the major
wheezing conditions is provided in Table xxx

82
Figure x. Algorithmic approach to young children presenting with wheeze.
*Therapeutic benefit from asthma medications is poor for those 1-2 years of
age and usually absent in the first year; ICS, inhaled corticosteroids

83
Table xx Summary of the most common wheezing conditions in young children (continues on next page)
Condition Estimated Clinical signs Investigation Expected Management
incidence in clinical course
children
Viral wheezing (these Very common, Wheeze No specific 60% will Trial salbutamol if >1 year
include a spectrum of especially in the associated with investigations outgrow of age and continue
viral LRTIs that are not first 2 years of life respiratory tract Nasal samples sent for wheeze by 6 only if effective
always clearly 50% of children infections virology usually do not years Supportive care involving
separated e.g. viral will have at least May be singular or change clinical A further 15% monitoring adequate
LRTI/recurrent viral- one wheezing recurrent management but acquire fluid intake (>50% of usual
induced episode Bronchiolitis (usually isolation of RSV in infants wheezing after intake) and for signs of
wheeze/bronchiolitis – in children <2 is highly suggestive of 6 years increasing respiratory
management of years) manifests bronchiolitis After 7–8 years, distress
episodes is identical and with fine crackles only 1 in 5 will
the distinction is +/– wheeze on outgrow it
sometimes arbitrary) auscultation

Asthma 15–20 % of the Wheeze on a Spirometry with Usually Exacerbations:

paediatric regular basis bronchodilator expected to Regular salbutamol (as
population Some will have response may be be lifelong but per asthma guidelines)
persistent/interval possible in children ≥5 clinical courses and consider oral
symptoms years of age in can vary prednisolone for up to 5
between episodes experienced widely days
of viral wheeze laboratories between Regular preventer usually
(cough and/or individuals indicated

84
 wheeze at night or
with exercise)

Table xx Summary of the most common wheezing conditions in young children (continued)

Condition Estimated Clinical signs Investigation Expected Management

incidence in clinical course
children

Airway malacia (airways 1 in 2100 Usually present Bronchoscopy usually Majority Treatment rarely required
floppiness): either soon after the diagnostic but not outgrow it by
tracheomalacia or neonatal period necessary in most age 2 years If there are worsening
bronchomalacia with wheeze, cases symptoms or failure to
stridor, cough and Secondary thrive, specialist referral is
rattling; children PBB can indicated
are usually well occur,
and often labelled presumably
as ‘happy from poor
wheezers’ cough
clearance

85
Protracted bacterial Probably Chronic wet Bronchoscopy may Majority 2–6 week course of
bronchitis (PBB) common, but cough (typically >4 assist diagnosis, but resolve with 1– antibiotics:
exact weeks). usually unnecessary 2 courses of
incidence Concurrent antibiotics commonly
unknown wheeze and/or Radiological findings amoxicillin/clavulanic
rattly breathing is usually normal or non- acid (approximately 20
common specific mg/kg/dose twice daily)

86
Is it asthma or viral wheeze? Which children outgrow this
phenomenon?

Key po ints
 Determining the cause of wheeze in young children can be
difficult and sometimes is determined only following a trial of
treatment.
 Parents’ description of wheeze can be inaccurate and often
needs elaboration or confirmation with impersonation or
video recordings.
 Asthma is very common but other causes are also common
and worth considering in the event of poor efficacy of asthma
treatment.
 A diagnosis of protracted bacterial bronchitis should be
considered for children with >4 weeks of continuous wet
cough.

87
BRONCHIOLITIS

Introduction
Bronchiolitis is a clinical diagnosis, based on history and
examination. It typically begins with an acute upper respiratory
tract infection followed by onset of respiratory distress and fever.
Illness usually resolves without intervention in 7 – 10 days, with
peak severity two to three days post onset.

Diagnosis

History
History should include:

 recent respiratory symptoms

 feeding including:
o duration of feeds (feeding more difficult with more
severe illness)
o breast feeding refusal
 underlying medical conditions including chronic lung disease,
congenital heart disease and chronic neurological conditions
 chromosomal abnormalities including Trisomy 21
 prematurity
 post-natal exposure to cigarette smoke

Symptoms and signs

 cough
 tachypnoea
 retractions
 diffuse crackles or wheeze on auscultation

88
Table xx. Examination

Assessment of severity of acute bronchiolitis

Mild Moderate Severe

Behaviour Normal Some/intermitten Increasing

t irritability irritability and/or
lethargy, fatigue

Respiratory Normal – mild Increased Marked increase

rate tachypnoea or decrease

Use of Nil to mild Moderate chest Marked chest

accessory chest wall wall retractions wall retractions
muscles retraction Tracheal tug Marked tracheal
Nasal flaring tug
Marked nasal
flaring

Oxygen SpO2 >92% SpO2 90-92% SpO2 <90%

saturations May not be
in room air corrected by O2

Apnoeic None May have brief May have

episodes apnoea increasingly
frequent or
prolonged
apnoea

Feeding Normal May have Reluctant or

difficulty with unable to feed
feeding or
reduced feeding

89
When to admit
When assessing a child in a secondary care setting, admit them
to hospital if they have any of the following:
 apnoea (observed or reported)
 persistent oxygen saturation of less than 92% when breathing
air
 inadequate oral fluid intake (50–75% of usual volume, taking
account of risk factors (and using clinical judgement)
 persisting severe respiratory distress, for example grunting,
marked chest recession, or a respiratory rate of over 70
breaths/minute.

When deciding whether to admit a child with bronchiolitis, take

account of any known risk factors for more severe bronchiolitis
such as:
 chronic lung disease (including bronchopulmonary dysplasia)
haemodynamically significant congenital heart disease
 age in young infants (under 3 months)
 premature birth, particularly under 32 weeks
 neuromuscular disorders
 immunodeficiency.

When deciding whether to admit a child, take into account

factors that might affect a carer's ability to look after a child with
bronchiolitis, for example:
 social circumstances
 the skill and confidence of the carer in looking after a child
with bronchiolitis at home. Confidence in being able to spot
red flag symptoms
 distance to healthcare in case of deterioration.

Consider seeking senior emergency/paediatric advice for infant

with moderate disease

Seek senior advice or refer for a child with severe bronchiolitis.

90
Risk factors for se vere disease
 gestational age less than 37 weeks
 chronological age at presentation less than 10 weeks
 chronic lung disease
 congenital heart disease
 chronic neurological conditions
 failure to thrive
 Trisomy 21
 post-natal exposure to cigarette smoke
 breast fed for less than 2 months

Differe ntial diagnoses

Whilst bronchiolitis is the most common cause of respiratory
distress in infants, less common diagnoses, or dual diagnoses must
be considered in all children.

Table 3. Differential diagnoses

Less common causes of respiratory distress in infants

Respiratory  bacterial pneumonia, including

pertussis
 aspiration of milk/formula or
foreign body
 tracheo/bronchomalacia

Other  congestive cardiac failure

 sepsis
 intrathoracic mass
 allergic reaction

91
92
CONGENITAL CARDIAC DISEASE

Caution
Consider cardiac disease presenting with congestive cardiac
failure in infants with no precipitating viral illness, hypoxia out of
proportion to severity of respiratory disease and/or presence of
abnormal or unequal peripheral pulses, cardiac murmur or
hepatomegaly.

Investigations
Investigations are not routinely recommended. Chest X-rays may
lead to unnecessary antibiotic treatment. The primary treatment
of bronchiolitis is supportive. This involves ensuring appropriate
oxygenation and maintenance of hydration.
Seek urgent paediatric critical care advice for infants with any of
the following:
 significant or recurrent apnoea
 persistent desaturations
 severe disease who are failing to improve with initial treatment

Management
Treatment

Children are often more settled if comfort oral feeds are

continued.

93
Table xx. Initial management (continues on next page)

INITIAL MANAGEMENT
The main treatment of bronchiolitis is supportive.
This involves ensuring appropriate oxygenation and fluid intake, and minimal handling

Mild Moderate Severe

Likelihood Suitable for discharge Likely admission, may be able to be Requires admission and consider
of admission discharged after a period of need for transfer to an appropriate
Consider risk factors
observation children’s
Management should be discussed facility/PICU
with a local senior physician Threshold for referral is
determined by local capacity but
should be early

Observations Adequate assessment in ED prior to One to two Hourly (not continuous) Hourly with continuous
Vital signs discharge (minimum of two cardiorespiratory (including
Once improving and not requiring
(respiratory rate, recorded measurements or every oximetry) monitoring and close
oxygen for 2 hours discontinue
heart rate, four hours) nursing observation
oxygen saturation monitoring
O2 saturations,
temperature)

94
95
Table xx. Initial management (continued)

Mild Moderate Severe

Hydration/nutrition Small frequent feeds If not feeding adequately (less If not feeding adequately
than 50% over 12 hours), (less than 50% over 12 hours), or
administer NG hydration unable to feed, administer NG
hydration

Oxygen Nil requirement Administer O2 to maintain Administer O2 to maintain

saturation/oxygen saturations greater than saturations greater than
requirement or equal to 90% or equal to 90%

Once improving and not requiring

oxygen for 2 hours discontinue
oxygen saturation monitoring

Respiratory Begin with NPO2 Consider HFNC or CPAP

support
HFNC to be used only if NPO2 has
failed

96
Table xx. Initial management (continued)

Mild Moderate Severe

Disposition/ Consider further medical review if Decision to admit should be Consider escalation if severity does
escalation early in the illness and any risk supported by clinical assessment not improve.
factors are present or if child (including risk factors), social and
Consider ICU review/ admission or
develops increasing severity after geographical factors, and phase
transfer to local centre with
discharge of illness
paediatric HDU/ICU capacity if:
 Severity does not improve
 Persistent desaturations
 Significant or recurrent
apnoea associated with
desaturations
 Has risk factors

Parental Provide advice on the expected Provide advice on the expected Provide advice on the expected
education course of illness and when to return course of illness and when to return course of illness
(worsening symptoms and inability (worsening symptoms and inability
to feed adequately) to feed adequately)

97
Oxyge n and respiratory support
Administer oxygen for children with saturations persistently below
the target oxygen saturations (SpO2) ≥92%

Table xx. Low flow oxygen

Low flow oxygen for infants with bronchiolitis by method of
delivery
Nasal prongs Hudson mask
Maximum flow Commence at a minimum flow rate of 4
rate of 2 L/min L/min to ensure adequate delivery if
oxygen requirement is greater than 2 L/min

High flow nasal c annula oxyge n (HFNC) Therapy

Consider HFNC therapy in infants with bronchiolitis who are
hypoxic (SpO2 <92%) with moderate to severe work of breathing.
HFNC therapy is not recommend for infants without hypoxia.
Continuous positive airways pressure (CPAP)
Nasal CPAP therapy for infants with bronchiolitis can also used.

Monitoring
Continuous pulse oximetry is recommended for hypoxic infants
or unstable infants receiving oxygen.

Hydratio n/nut rition

 Give small frequent feeds for infants with mild bronchiolitis
 Consider nasal saline drops prior to the time of feeding
 Suction of the nares may assist feeding in infants with
moderate distress
Caution – Deep suctioning of the nasopharynx is not
recommended as may cause oedema and irritation of the
upper airway resulting in increased length of illness.
 NGT insertion is highly recommended for infants on HFNC.
Advantages include:
o gastric decompression
o ability to feed without interrupting HFNC
98
 o avoid potential for worsening of respiratory symptoms
during feeding
 NG or IV hydration is recommended for infants with moderate
-severe bronchiolitis who are feeding inadequately (less than
50% over 12 hours)
 if using IV route, isotonic IV fluids (0.9% sodium chloride with
glucose, or similar) are recommended
 the volume of fluids required to maintain hydration is unclear

Treatme nts NOT recommended

 beta 2 agonists (e.g. Salbutamol) regardless of a
personal/family history of atopy
 corticosteroids
 adrenaline (nebulised, IM, or IV) except in peri-arrest or arrest
situation
 hypertonic saline
 antibiotics
 antivirals
 deep nasal suction
 chest physiotherapy

When to refer
Immediately refer children with bronchiolitis for emergency
hospital care if they have any of the following:
 apnoea (observed or reported)
 child looks seriously unwell
 severe respiratory distress, for example grunting, marked chest
recession, or a respiratory rate of over 70 breaths/minute
 central cyanosis
 persistent oxygen saturation of less than 92% when breathing
air.
 difficulty with breastfeeding or inadequate oral fluid intake
(50–75% of usual volume, taking account of risk factors and
using clinical judgement)
 clinical dehydration.
99
When to discharge
Consider discharge for the following infants:
 is clinically stable
 has maintained oxygen saturation over 92% in air for 4 hours,
including a period of sleep.
 feeding adequately
 parent/caregiver can safely manage the infant at home
(consider time of day, parent/carer comprehension and
compliance, access to transport and distance to the local
clinic).

Key safety informat ion for looking after a child at

home
Provide key safety information for parents and carers to take
away for reference for children who will be looked after at
home. This should cover how to recognise developing 'red flag'
symptoms:
 Worsening work of breathing (for example grunting, nasal
flaring, marked chest recession)
 Fluid intake is 50–75% of normal or no wet nappy for 12 hours
 Apnoea or cyanosis
 Exhaustion (for example, not responding normally to social
cues, wakes only with prolonged stimulation).
 That people should not smoke in the child's home because it
increases the risk of more severe symptoms in bronchiolitis
 How to get immediate help from an appropriate professional
if any red flag symptoms develop
 Arrangements for follow-up if necessary.

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associated with hospitalizations for acute respiratory illness in
young US children. J Infect Dis 2011;204:1702–10. Search
PubMed
Miller EK, Williams J V, Gebretsadik T, et al. Host and viral
factors associated with severity of human rhinovirus-
associated infant respiratory tract illness. J Allergy Clin
Immunol 2011;127:883–91. Search PubMed

Lee W-M, Lemanske RF, Evans MD, et al. Human rhinovirus

species and season of infection determine illness severity.
Am J Respir Crit Care Med 2012;186:886–91. Search PubMed
Wisdom A, Kutkowska AE, McWilliam Leitch EC, et al.
Genetics, recombination and clinical features of human
rhinovirus species C (HRV-C) infections; interactions of HRV-C
with other respiratory viruses. PLoS One. 2009;4:e8518. Search
PubMed

103
104
ACUTE EXACERBATION OF ASTHMA

Definition and clinical prese ntation

Asthma is a chronic inflammatory disorder of the airways,
associated with airway hyperresponsiveness leading to recurrent
wheezy episodes, breathlessness, chest tightness and persistent
cough.

Diagnostic crite ria

 Episodes of breathlessness
 Chest tightness
 Wheezing
 Persistent cough
 Airflow obstruction, variability and reversibility by peak flow
measurement or spirometry

Table Assessment of Asthma Severity

Sign Mild to Severe Life-threatening
Moderate exacerbation exacerbation
exacerbation
Sa02 92-95% < 92% < 90%

RR
< 50/min > 50/ min
2-5 years Variable
< 30/min > 30/ min
> 5 years

HR 100-120/min > 120/min Variable

Effort of Use of accessory Poor respiratory
Normal
breathing neck muscles effort

Expiratory and
Wheeze Expiratory Silent chest
inspiratory

Speaking with
Speech Normal Unable to speak
difficulty

Level of Fully
Agitated Confused
consciousness conscious

If no pulse oxymeter is available, check for cyanosis.

105
Table XX . Manage ment
Mild to Moderate Severe exacerbation Life-threatening
exacerbation asthma
Salbutamol High flow Oxygen Admit to PICU and
4-10 puffs via MDI 5-10 L/min. treat as for severe
and spacer every 20 Salbutamol as for mild exacerbation
minutes for one hour to moderate
then every 30 minutes exacerbation
to 4 hourly, In addition:

Add ipratropium Do FBC, ESR, U&Es

OR
bromide via spacer or blood culture, ABG
Salbutamol nebuliser: if available
nebulisations
125 mcg < 1 year
2.5 mg < 2 years
250 mcg if 1-5 years
5 mg > 2 years
500 mcg >5 years IV 50% magnesium
4-6 hourly sulphate 40 mg/kg
over 20 min (Max 2
Start prednisolone
grams)
early 2 mg/kg Give prednisolone
Diluted 1:5 in 0.9%
(Max 40 mg) PO If not improving
Saline
PO OD for 3 days or vomiting

IV hydrocortisone SC adrenaline
Discharge if
4 mg/kg 6 hourly 1:1000
improving
0.01 mg/kg
(Max 0.5 mg)
IV aminophylline
Otherwise admit
5 mg/kg stat over
and continue with
20 min, then If not improving,
regular salbutamol
infusion at 1 Prepare for
mg/kg/hour intubation and
mechanical
ventilation
 Admit to PICU
 Regular reviews
 Footnote on
aminophylline
and adrenaline

106
NOTE. Aminophylline and adrenaline should never be used as
first line treatment for acute exacerbation of asthma, except in
situations where no better alternative is available.

Disc harge plan

 Patient can be discharged when stable on 4 hourly
salbutamol inhalations.
 To complete 3 days course of prednisolone.
 Patient/parent education to be done on the ward.
 Patients with mild exacerbation of asthma can be reviewed
at the local clinic.
 Those with moderate, severe or life-threatening exacerbations
should be booked for review in the asthma clinic.
 Reliever: as-needed rather than routinely
 Controller: Higher dose for short term (1-2 weeks) or long term
(3 months) depending on the background of the
exacerbation.
 Risk factors: Check and correct modifiable risk factors
including inhaler technique.
 Written action plan.

Refere nces
British Thoracic Society. Management of Asthma
Guidelines, April 2004.
Papadopoulos NG and Kalobatson A. Respiratory viruses in
childhood asthma. Curr Opin Allergy Clin Immunol, 2007; 7(1)
91-95
Tomlinson R. Postcard from Africa: Hospital management of
asthma. Arch dis child, 2002; 87:356
AL-Hajjaj MS. Bronchial asthma in developing countries: A
major social and and economic burden. Annals of Thoracic
Medicine, April-June 2008, vol 3, 2:39
Fischer GB and Camargos PAM. Paediatric asthma
management in developing countries. Paediatric respiratory
reviews, 2002, 3: 285-291

107
Busse VW and Lemanske RF. Asthma. New Engl Jour of Med,
February 2001, Number 5, vol 344:350-362
GINA. Global strategy for asthma management and
prevention. Updated 2018 report.

108
PNEUMONIA
Pneumonia is an important cause of morbidity and mortality in
developing countries
Both bacteria and viruses are important causes of pneumonia

Bacteria are the most common cause of pneumonia in HIV-

infected children

Community-ac quire d pneumonia

 Pneumonia acquired outside the hospital settings
 In younger children, viral organisms are the common
causative agents
 Streptococcal pneumoniae is the most likely cause in older
children
 Mycoplasma pneumoniae should be considered in school
going children and adolescents

Clinical presentation
 Fever >38.50 C
 Tachypnoea, tachycardia
 Subcostal and intercostal recession
 Crackles
 Abdominal pain (referred pleural pain)

Age Cut-offs for fast breathing

< 2 months 60 breaths/min or more

2 – 12 months 50 breaths/min or more

12 Months - 5 Years 40 breaths/min or more

109
Indications for admission
 Cyanosis, Sp O2 < 92%
 Increased respiratory rate
 Subcostal recession
 Intermittent apnoea, grunting (infants)
 Poor feeding
 Convulsions
 Restlessness or agitation
 Signs of dehydration
 Unconscious or lethargic
 Capillary refill time >3 seconds

Investigations
 Chest x-ray
 FBC, ESR
 Urea, electrolytes (may have hyponatremia owing to possible
SIADH), and creatinine
 Blood culture
 If able to produce good sputum (send for gene xpert)
 Nasopharyngeal aspirates (send for gene xpert)
 Arterial blood gases (if available)

Management
 General measures
o Oxygen by nasal cannula or mask
o IV fluids if required should be < 2/3 of requirements (risk
of SIADH in hypoxic children)
o Antipyretic and analgesia as indicated
o Monitor vital signs

 Antibiotics therapy
o 0-3 months
 Benzyl penicillin 50,000 IU/kg/dose every 6 hours and
gentamicin 7.5 mg OD or BD (in divided doses)
110
 o More than 3 months
 Benzyl penicillin 50,000 IU/kg/dose every 6 hours
o Ceftriaxone (50-80 mg/kg/dose OD) or cefotaxime (50
mg/kg QDS) should be considered if no improvement
within 48 hours
o In infants with HIV infection or exposure, PCP therapy
with high dose IV/PO cotrimoxazole (20 mg/kg/day of
trimethoprim) should be included
o In suspected staphylococcus pneumonia, add IV
Cloxacillin
o Macrolides should be considered in school-going
children and adolescents who do not improve on 1 st line
treatment
 Erythromycin
1 month – 2 years: 125 mg QID
2 – 8 years: 250 mg QID
8 – 18 years: 250-500 mg QID
 Azithromycin
6 months-2years: 10 mg/kg OD
3 – 7 years: 200 mg
8 – 11 Years: 300 mg
12 – 14 years: 400 mg
>14 years: 500 mg

Refere nces
British Thoracic Society Guidelines on the management of
community-acquired pneumonia in childhood. Thorax 2002;
57:i1-i24
WHO. Management of children with pneumonia and HIV in
low resource settings. Report on consultative meeting
Harare, Zimbabwe, 30-31 January 2003. Geneva
WHO. Hospital care for children. Guidelines for the
management of common illnesses in low resource settings.
2005. Geneva

111
Bedside Clinical Guidelines Partnership in association with
partners in paediatrics Paediatric Guidelines. 2016-18.

112
CENTRAL NERVOUS SYSTEM

COMA
Definition

A state of deep unarousable unconsciousness with total loss of

awareness of self and environment. Consciousness is awareness
of oneself and surroundings in a state of wakefulness, altered by
disease, poisoning or trauma.

Causes

Coma with fever Coma without fever

 Meningitis/sepsis  Metabolic disorder

 Cerebral malaria  Hypertensive
 Viral meningo-encephalitis encephalopathy
 Post-ictal state
 Non-Convulsive status
epilepticus
 Stroke
 Poisoning/intoxications
 Mass brain lesions with
raised ICP

Focused clinic al history

 Child’s health and activity in the last 5 days (preceding viral
illness)
 Any history of fever
 Any chronic condition (DM, SCA, epilepsy)
 Time of last meal
 Pre-existing neurological disability
 Recent trauma

113
General physical examination
 Check vital signs- for signs of increased intracranial pressure(
bradycardia, hypertension, respiratory rate/pattern (Cheyne
stokes breathing)- Cushing triad
 Head – Full AF
 Eyes – Pupillary size and reaction to light and position (CN III,IV
and VI palsies)
 Neck – Rigidity
 Odour – Metabolic disorders, poisoning
 Abdomen – Enlarged liver associated with hypoglycaemia
 Skin – Rash, trauma, haemorrhage

Primary assessment /re suscitation

A Airway

B Effort of breathing: Recessions, RR, grunting, flaring,

use of accessory muscles.
Effect of breathing: HR, skin colour, mental state,
cyanosis
Pattern of b breathing: Cheyne- stokes

C Circulation: Pulse volume, pulse rate, capillary refill

time, BP
D Disability: A Alert
V Verbal
P Pain
U Unresponsive
Degree of coma (see below), posture
(decorticate, decerebrate, hemiparesis),
pupils, seizures
E Exposure: Temperature, rash, evidence of poison

114
Degree of coma
This can be measured using:

115
Glasgow Coma Scale
> 1 Year < 1 year Score
Spontaneously Spontaneously 4
To verbal command To shout 3
EYE OPENING
To pain To pain 2
No response No response 1
Obeys Spontaneous 6
Localises pain Localises pain 5
Flexion withdrawal Flexion withdrawal 4
MOTOR RESPONSE
Flexion-abnormal (decorticate rigidity) Flexion-abnormal (decorticate rigidity) 3
Extension (decerebrate rigidity) Extension (decerebrate rigidity) 2
No response No response 1
> 5 years 2 – 5 years 0 – 23 Months
Oriented Appropriate words / phrases Smiles/ cools appropriately 5
Disoriented/confused Inappropriate words Cries and is consolable 4
Inappropriate words Persistent cries and screams Persistent inappropriate crying and/or 3
VERBAL RESPONSE
screaming
Incomprehensible sounds Grunts Grunts, agitated, and restless 2
No response No response No response 1
TOTAL PAEDIATRIC GLASGOW COMA SCALE (3 – 15):
116
Investigations
 Blood glucose
 Malaria parasite slide/RDT
 FBC, Renal function tests, Electrolytes (calcium, phosphate,
Mg), blood culture,
 LFTs
 Blood/
 Urine toxicology
 CSF studies (if not contraindicated)
 EEG
 Imaging studies
o X-rays in order to localise infection ( e.g. Chest x-ray to
r/o disseminated TB)
o Neuro-imaging based on clinical findings and
judgement .

Management
 Pay meticulous attention to Airway, Breathing, Circulation and
Disability
 Check RBS (if not available, give 5mls/kg of 10% dextrose IV)
 Treat for raised ICP, if present- See next chapter
 Nursing care (skin care, oral hygiene, eye care, feeds, fluid
balance)
 Further treatment depends on the provisional diagnosis

Refere nces
Behrman RE et al. Nelson Textbook of Pediatrics, 18 th edition,
2007, Saunders, Elsevier
James HE. Emergency of acute coma in children, Pubmed,
1993 Sep 1; 48(3):473-8
Donald AT. Coma in the Pediatric Patient: Evaluation and
Management. Indian Journal of Pediatrics, Volume 61,
Number 1, 13-26
Pearl PL. Neuro-logic: A primer on localisation, 2014, Demos
Medical Publishing, New York.

117
118
INTRACRANIAL PRESSURE

Definition
 Intracranial pressure (ICP) is the pressure within the cranium
and is measured in mm Hg. Normal values are 5-15 mmHg.
 Intracranial hypertension (or raised ICP) is defined as sustained
ICP levels above 20mmHg.

Causes
 Traumatic Brain Injury, with mass effect
 Large vessel ischemic stroke (Carotid or MCA occlusion)
 Cytotoxic cerebral oedema with mass effect.
 Intracranial haemorrhage with mass effect.
 Hydrocephalus
 Diffuse cerebral oedema(hepatic failure,
cerebritis/encephalitis/meningitis)
 Jugular venous obstruction or elevated right sided cardiac
venous pressure
 Brain neoplasms causing mass effect or vasogenic oedema
 Idiopathic oedema

Focused history
 Pain/vomiting with  Nausea
valsalva manoeuvres  Vomiting
 Headache

Examinatio n
 Papilledema in awake patients(may proceed to stupor and
coma over time)
 Bradycardia, hypertension and cheyne stokes breathing in
comatose patients.
 Papillary dilation, extensor or flexor posturing and Cheyne
stokes breathing, as a result of acute stupor or coma
 Nuchal rigidity
 Retinal haemorrhages
 Bulging fontanel and sun-setting eyes in infants

119
 CN III (often ipsilateral anisocoria), IV, or VI palsies (VI very
common!)

Management

CLINICAL SIGNS OF RAISED ICP PRESENT CT imaging and liaison

This is a medical emergency with Neurosurgical /
Anaesthetic team if
Document findings; start continuous
available
cardiopulmonary monitoring and alert
intensive care/senior doctor to attend
urgently.

MAXIMAL MEDICAL THERAPY

1. ABCDE assessment and stabilisation: REMINDERS:-

o If head/neck injury is suspected, protect and stabilise
the C-spine as a priority
o If the airway is at risk if GCS <8. Use airway control with
airway manoeuvres, adjuncts as appropriate
o Breathing – ventilate as required, give oxygen
o Circulation – maintain mean arterial pressure with
vasopressors if required
2. Elevate head of bed by 15 – 30 degrees.
3. Maintain normothermia (36.5oC – 37.5oC).
4. Consider hyperventilation if available, with close monitoring
(note – effects only lasts for minutes).
5. Give 20% mannitol if available, with close monitoring.
6. Give 3% saline if available, with close monitoring.
7. If no signs of improvement, discuss with senior staff to advise
on further management (e.g. sedation, intubation,
ventilation).

Dose for mannitol 20% (APLS 2018)

250 – 500 mg/kg single dose, IV infusion over 30 min (1.25 –
2.5 ml/kg of 20% solution)

Dose for 3% saline (APLS 2018)

3 – 5 ml/kg IV over 15 minutes

120
Refere nce

Freeman WD. Management of intracranial pressure.

Continuum (Minneap Minn) 2015;21(5 Neurocritical
Care):1299Y1323

Advanced Life Support Group, 2018. Advanced Paediatric

Life Support: A Practice Approach to Emergencies (6 th ed)
p89-98.

121
SEIZURES

First unpro voked seizure

Definitions (ILAE)

An epileptic seizure is a transient occurrence of signs and/or

symptoms due to abnormal excessive or synchronous neuronal
activity in the brain
Epilepsy is defined as a chronic disorder of the brain
characterized by an enduring predisposition to generate
epileptic seizures, and by the neurobiological, cognitive,
psychological, and social consequences of this condition. The
definition of epilepsy requires the occurrence of at least one
epileptic seizure. The definition of epilepsy is usually practically
applied as:
 Having two unprovoked seizures >24 h apart.
 A patient with a first unprovoked seizure after a remote brain
insults (such as a stroke, CNS infection, trauma) with a
recurrence risk of further seizures comparable to the general
recurrence (greater than 60%) risk for further seizures after two
unprovoked seizures over the next ten years.
 A diagnosis of an Epilepsy syndrome

First unprovoked seizure: A focal or generalized seizure, including

multiple seizures within 24 hours with full recovery of
consciousness between seizures, without a clear provoking
factor such as head trauma immediately preceding the seizure,
CNS infection, or other known acute cause. Also to be excluded
are neonatal seizures (in children <28 days old), status
epilepticus, and febrile seizures.

Careful history and Examinatio n

Specifically evaluate for risk factors of epilepsy including:
 Prior seizures
 Family history of epilepsy
 Any concerning historical factors for provoked seizures-
including dehydration, vomiting, diarrhoea, toxicology
exposure

122
 abnormality on neurologic exam
 concern for other neurologic disorders

Investigations
 Laboratory testing should be performed based upon
individual clinical circumstances.
 Serum glucose, sodium, calcium and magnesium in particular
should be considered if concern for electrolyte abnormalities
per history
 Toxicology screening should be considered in any paediatric
patient if any question of drug exposure or substance abuse
 EEG should be considered in cases of first time seizure to
assess for underlying epileptiform abnormalities and determine
risk of future seizures (e.g. diagnosis of epilepsy) when the
study is feasible to obtain and clinical management is in
question. Findings should be interpreted with the clinical
history to make a determination if the child has epilepsy. A
normal study does not exclude epilepsy. EEG testing is not
necessary to diagnose epilepsy or initiate antiepileptic
medication.
 Emergent neuro-imaging should be obtained in any child with
o persistent unresponsiveness
o Lack of return to baseline within a few hours
o Persistent Todd’s paresis (transient weakness or paralysis
of part or all of the body after a focal-onset seizure) that
is not resolving within a few hours.

As timing is essential, the recommended study would be CT

unless MRI is feasible within hours.
If the child is back to baseline, a non-urgent MRI/CTI (depending
on availability and clinical suspicion- e.g. CT should be
performed to rule out neuro-cystercercosis) should be
considered for any child who has
 A focal seizure
 Focal EEG findings

123
 Significant cognitive or motor impairment without known
aetiology
 Other unexplained abnormality on neurological exam
particularly if unresponsiveness to AED treatment or
progressively worsening development is noted.
A diagnosis of epilepsy should be made if:
 A child with 1 unprovoked seizure presents with
o an abnormal EEG
o structural abnormality on neuro-imaging
o clear neurologic abnormality on examination suggestive
of an underlying neurologic abnormality as the risk of
seizure recurrence within a 10 year period is estimated as
being higher than 60%.
 A child has 2 or more unprovoked seizures greater than 24
hours apart.
If epilepsy is diagnosed, consideration for antiepileptic treatment
should be made based upon the clinical circumstances,
All children should be followed up after the first unprovoked
seizure to see if there have been any recurrent events and
reassess for risk of epilepsy, even if EEG and other evaluations
were normal.

124
 First unprovoked seizure

Careful history and physical exam;

laboratory testing if clinically indicated

Child back to baseline? NO

YES
Investigate for other
clinical causes,
Assess for risk of epilepsy by clinical including non-
evaluation and available diagnostic convulsive status
testing (EEG and if indicated non urgent epilepticus (see
neuroimaging) status epilepticus
protocol )

Start appropriate antiepileptic

medication when clinically indicated
(e.g. abnormal exam, risk factor by
history or abnormal EEG or MRI findings)

All children (whether medication is

initiated or not) should have follow up
within 3 months to reassess

125
Treatme nt

Guidelines for first line anti-epilept ic drug choice

Age < 2 years Age > 2 years

Infantile YES YES

High dose steroid 1) LVT HIV+/on ART
spasms? protocol 2) VPA meds?
Refer to specialist
NO NO

HIV+/on ART YES 1) LVT Focal, generalised,

meds? 2) Use VPA with mixed, or unable to
caution (Risk determine seizure
with metabolic type?
NO
dz)

Focal, generalised,
mixed, or unable to
determine seizure
type? Focal Generalised, mixed,
or unable to
determine seizure
type?
Focal Generalised, mixed,
or unable to
determine seizure
type?
1) CBZ 1) VPA
2) VPA 2) PB (caution if
3) LVT behavioural
4) PB (caution if disorder)
1) CBZ 1) PB
behavioural
2) PB 2) LVT
disorder)
3) LVT 3) VPA (Risk with
metabolic dz)

CBZ-carbamazepine, PB-phenobarbital, VPA- valproic acid, PHT- phenytoin, LVT-

levetiracetam

126
Refere nces
Hirtz, D, Ashwal, S, Berg, A, Bettis, D, Camfield, C, Camfield,
P, Crumrine, P,Elterman, R, Schneider, S. and Shinnar, S.
Practice parameter: Evaluating a first nonfebrile seizure in
children: Report of the Quality Standards Subcommittee of
the American Academy of Neurology, the Child Neurology
Society, and the American Epilepsy Society. Neurology
2000;55;616-623
World Health Organization Updated Guideline on Paediatric
Emergency Triage, Assessment and Treatment Care of
Critically Ill Children. Geneva, World Health Organization,
2016.
WHO, Department of Mental Health and Substance Abuse.
Mental Health Gap Action Programme (mhGAP)
Intervention Guide version 2.0. October 2016.
Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official
report: a practical clinical definition of epilepsy. Epilepsia
2014;55:475–82.

127
STATUS EPILEPTICUS

Conceptual de finition (IL AE 2015)

Status epilepticus is a condition resulting either from the failure of
the mechanisms responsible for seizure termination or from the
initiation of mechanisms, which lead to abnormally, prolonged
seizures (after time point t1 - 5min) and can have long-term
consequences (after time point t2 – 30min), including neuronal
death, neuronal injury, and alteration of neuronal networks,
depending on the type and duration of seizures.

Classic al Definition:
Continuous convulsion lasting longer than 20 to 30 mins or the
occurrence of serial convulsions between which there is no
return of consciousness.

Operatio nal Definitio n

Seizure lasting more than 5 min or recurrence of two or more
seizures, between which there is incomplete recovery of
consciousness
Every child who presents for clinical care while having a seizure
must be treated as status epilepticus.

Causes
 Febrile seizures
 Known epileptic who is non-compliant to treatment, is on
erratic supply of drugs, had sudden withdrawal of
anticonvulsants, is sleep deprived or has an inter-current
infection
 Initial presentation of epilepsy
 CNS infections
 Congenital malformations of the brain (lissencephaly,
shizencephaly)
 IEMs especially in neonates
 Electrolyte abnormalities – hypocalcaemia, hypoglycaemia
 Drug intoxication – amphetamines, cocaine, phenothiazines,
theophylline, TCAs

128
 Others – Reye’s syndrome, lead intoxication, extreme
hyperpyrexia, brain tumour.

Investigations
 FBC and DC
 Random Blood Sugar
 Electrolytes & creatinine
 LFTs
 Calcium, magnesium, phosphate
 Gas analysis
 Lactate
 CSF studies (after brain CT)

Treatme nt
There are 4 goals of therapy:
 Ensure adequate vital signs, systemic and cerebral
oxygenation
 Terminate seizure activity
 Prevent seizure recurrence
 Establish the diagnosis and treat the underlying cause if
possible

First steps in management include stabilizing the child-

ensure airway is open and circulation is stable. Administer
oxygen. Place an IV line if possible.
16. Immediately treat first with diazepam (or if available,
Lorazepam).

o If IV access is available, use 0.2-0.3mg/kg of diazepam

IV (or 0.1mg/kg of Lorazepam) and there after give
glucose (5ml/kg of 10% dextrose)
o If no IV access, administer diazepam 0.5mg/kg rectally.
Diazepam should NOT be administered intramuscularly.

17. 5 minutes:

129
o Administer a 2nd dose of diazepam. DO NOT EXCEED 2
DOSES.
o Alternatively, after the 1st dose of diazepam is
administered, and there are limited resources for
respiratory support, consider proceeding to step 4

18. 10 minutes

o If the seizures do not stop and Phenytoin is available,

administer 20mg/kg IV x 1 (max 1gm), (dilute in normal
saline- 100mg in 50ml) given over 20minutes.
o This must be administered in a new IV (not in dextrose)
using a G22 cannula. Cardiorespiratory monitoring is
also indicated due to risk for arrhythmias and
hypotension.
o An additional 10mg/kg can be administered if seizures
continue 15 minutes after the first dose

19. If phenytoin is not available:

o Stable respiratory status: administer 15-20mg/kg over 20

minutes (maximum dose 1gm) of phenobarbital with
caution, very closely monitoring respiratory status.
o If respiratory status is not stable, provide available
respiratory support. If there is an option for mechanical
ventilation, emergently prepare to intubate the child if
needed as you administer phenobarbital. If there is no
mechanical ventilation available, choose one of the
following based upon medication availability:
 Levetiracetam 40mg/kg (max dose 1gm) IV (preferred)
or NGT
 Sodium valproate 20mg/kg (max dose 1gm) (only in
children over 2 years) IV if no other option consider
administer via NGT
 OR Phenobarbital 10mg/kg IV or IM x 1 and repeat the
dose as you monitor respiratory status closely.
20. If the seizures continue, use any of the other medication
options listed. Alternatively, if no other options are
available and there is no clear infection, consider a trial
130
 of high dose steroids of 30mg/kg daily
Methylprednisolone (max 1gm) x 3 days, followed by a
one-week oral prednisolone taper.

o If available, other treatment options for refractory status

epilepticus include midazolam infusion, ketamine,
propofol, pentobarbital coma, thiopental and other
inhaled anesthetics.

21. Evaluate (and treat as appropriate) for underlying cause

of seizure:

o Consider head CT, and request urgent EEG (if feasible) if

there is any concern for non-convulsive status
epilepticus.
o Review medication compliance, dose and formulation
for all children with prior Epilepsy diagnosis and re-dose
home medication if indicated

22. If the child has high risk of seizure recurrence (e.g. CNS
infection), maintain on antiepileptic medication until child
can be seen in follow up (within 3 months)
23. If no provoking factor for SE can be identified, and the child
does not have known epilepsy, proceed with investigations
as noted in first unprovoked seizure guidelines
24. Children with known epilepsy and on medication who
present in status epilepticus should have re-evaluation of
treatment plan and adjustment of medication prior to
discharge

25. All children who present in status epilepticus should be given

a rescue medication (e.g. rectal diazepam) and instructions
on seizure first aid.

See appendix X/next page

131
132
 START 1. Assess and stabilize as needed for breathing and
hemodynamic status. Administer oxygen.
Place the child on his/her side, loosen clothing and do not
place anything in the mouth.

5 MINUTES 2. Administer A Benzodiazepine

IV access If no IV access
Dextrose -5ml/kg of 10% PR Diazepam (0.5mg/kg).
dextrose.
Intranasal Lorazepam
IV Diazepam (0.2-0.3mg/kg). (0.1mg/kg, max 4mg).
IV Lorazepam (0.1mg/kg, Buccal/ intranasal
maximum dose 4mg) Midazolam (0.2mg/kg,
max 5mg).
Diazepam should never be
given IM intramuscularly.

10 MINUTES 3. If seizures persist, give 2nd dose of the benzodiazepine

15 MINUTES 3. If seizures persist,

Phenytoin available: Phenytoin unavailable:

Administer 15-20mg/kg IV x 1 Phenobarbital:15-20mg/kg over 20
(max 1gm) minutes (maximum dose 1gm) monitor
respirations OR
Levetiracetam 40mg/kg (max dose 2gm)
IV (preferred) or NGT OR
Sodium Valproate 20mg/kg (max dose
1gm) (only in children over 2 years) IV.
(If no other option, consider administering
via NGT)

If seizures continue, use one of the other medications (if available) OR if

Phenytoin is used, an additional 10 mg/kg Phenytoin (given over 30 min)
can be administered. It is essential to monitor for respiratory depression,
hypotension, and arrhythmia. Evaluate (and treat as appropriate) for
underlying cause of seizure

133
Refere nces
World Health Organization Updated Guideline on Paediatric
Emergency Triage, Assessment and Treatment Care of
Critically Ill Children. Geneva, World Health Organization,
2016.
WHO, Department of Mental Health and Substance Abuse.
Mental Health Gap Action Programme (mhGAP)
Intervention Guide version 2.0. October 2016.
Trinka, E. et al. A definition and classification of status
epilepticus--Report of the ILAE Task Force on Classification of
Status Epilepticus. Epilepsia. 2015.
Trinka, E et al. Pharmacotherapy for Status epilepticus. Drugs
(2015) 75:1499–1521
Birbeck et al, A clinical trial of enteral Levetiracetam for
acute seizures in pediatric cerebral malaria BMC Pediatrics,
(2019) 19:399

134
FEBRILE SEIZURES

Definition
Febrile seizures are seizures that occur in febrile children between
the ages of 6 and 60 months who do not have an intracranial
infection, metabolic disturbance, or history of afebrile seizures or
significant neurological impairment. Fever may not be detected
before the seizure, but it must be present at least in the
immediate post-acute period and may be the only symptom of
presenting illness.
 Simple febrile seizure
o Usually generalized tonic-clonic
o Lasting for a maximum of 15 min
o not recurrent within a 24-hour period.

 Complex febrile seizure

o More prolonged (>15 min)
o Maybe focal
o With/without associated post-ictal palsy (Todds paresis)
o And/or recurs within 24 hours

 Febrile seizures frequently occur in the contest of:

o Viral URTIs
o HHV6 infection
o Acute otitis media
o Malaria
o Genetic predisposition, e.g. autosomal inheritance
pattern in some families

Clinical features
 Core temperature to ≥ 38⁰c
 Generalized tonic- clonic, lasting up to 15 min
 Occurs once in 24 hrs (usually on first day of fever)
 For complex type – see above
 Recurrence is possible
 The vast majority are harmless, prognosis is good
135
Complete ge neral and ne urologic al examination
 Stabilize the child
 If the child arrives seizing to the facility, treat as status
epilepticus (see status epilepticus protocol)
 Check for focus of infections
 Check for possible signs and symptoms of meningitis (see
protocol for meningitis).
 NB: Lumbar puncture is not required in simple febrile seizures.
It should be considered in the following scenarios:
o Children under 18 months of age (other signs and
symptoms of meningitis might not be present or be very
subtle).
o Complex febrile seizures (should only be considered
after excluding need for neuro-imaging)
o Children who have received antimicrobials prior to
assessment
o Children not immunised against Haemophilius and
Pneumococcal species

Investigations
 Investigation should be done on individual basis to determine
the cause of fever - MPS, FBC, nasopharyngeal swab, urine
m/c/s, if no obvious focus is found.
 EEG and Neuroimaging are not routinely required for febrile
seizures
 The work-up of children with complex febrile seizures needs to
be individualized. This can include EEG and neuro-imaging
where there is a clear focality concerning for possible CNS
lesion.

Treatme nt
 Abort seizure - Diazepam IV 0.2 – 0.5 mg/kg slowly (can also
be given rectally 0.5 mg/kg) if duration more than 3-5 min.

136
 Supportive therapy – tepid sponging, nurse in semi-prone
position, ensure adequate airway.
 Assess for the cause of fever and treat appropriately -
Coartem, amoxyl...
 Antiepileptic medications are not indicated.
 Antipyretic should be used at the same dose and intervals
used for lowering fever during a febrile illness at optimal
dosing:

o Paracetamol at 10 to 15 mg/ kg/dose every 4-6 hours

(max 5 doses/day)
o Ibuprofen: 20-30 mg/kg/day in 3 doses.

 Families should be provided counselling on risk or recurrence

of febrile seizures (25-40%), risk of epilepsy (10% if complex
febrile seizures, similar to general population if simple febrile
seizures), and seizure first aid.

Refere nces
Kliegman R, Berhman R et al; Nelson Textbook of Pediatrics;
Saunders Elsevier; 18thed, 2007
Ghai OP, Paul V &Bagga A; Ghai Essential Paediatrics; CBS
Publishers; 7thed, 2009
Oxford Handbook of Paediatrics; Oxford University Press; First
ed; 2008.
British National Formulary for Children, Bmj Publishers; 2007

R.S. Fisher at al. Epilepsia. 2005;46:470-2)

R.S. Fisher at al. Epilepsia:1–8, 2014
Lowestein et al. Epilepsia. 1999;40 Suppl 1:S3-8
World Health Organization Updated Guideline on Paediatric
Emergency Triage, Assessment and Treatment Care of
Critically Ill Children. Geneva, World Health Organization,
2016.

137
WHO, Department of Mental Health and Substance Abuse.
Mental Health Gap Action Programme (mhGAP)
Intervention Guide version 2.0. October 2016.

138
PAEDIATRIC STROKE

Definition
A focal neurological deficit with an underlying vascular
pathology is defined as:

Stroke lasting > 24h

Transient ischaemic attack (TIA) lasting < 24h
Reversible ischaemic neurological lasting> 24 h but with full recovery
deficit (RIND)
“Stroke-like episode” Focal neurological deficit lasting
>24 hr with no obvious vascular
pathology, e.g. Brain tumour, brain
abscess.
Rare in childhood (1-3 / 100,000 per
year)

Paediatric stroke can be ischaemic (vessel spasm, stenosis,

dissection or vessel occlusion by thrombosis or embolism) or
haemorrhagic and occur at any age;
 Neonatal stroke:28 weeks gestation- 28 days post natal life
 Childhood stroke: Greater than 28 days -18 years

Note: SCD is the most common cause of stroke in Zambian

children.

139
Ischaemia ( Arterial or Venous)

Embolism Cyanotic CHD, Endocarditis

Large vessel stenosis SCD, Varicella, HIV, Homocystinuria

Vessel spasm CNS infections

SCD, Severe dehydration (cerebro-

venous-sinus thrombosis), Malignancies
(Leukemia)
Thrombocytosis, CNS infections, hyper
Thrombosis
coagulable states i.e Protein S or C
deficiency, Antithrombin III deficiency,
Lupus antibodies, Factor V Laiden, Renal
disorders i.e Nephrotic syndrome

Vessel dissection Trauma, Congenital Heart Disease

Moya Moya, Focal/transient arteriopathy,
Cerebral Arteropathy
Post varicella
Drugs i.e Aspiraginase

Syndromes Williams syndrome, Downs syndrome

Haemo rrhage
Low platelets Idiopathic thrombocytopaenic purpura
Bleeding disorder Coagulation factor deficiencies i.e Hemophilia
Vessel disorder A-V malformation, A-V fistulas, cerebral aneurysm,
Carvenous malforrmations
Trauma

Clinical features
 Hemiparesis
 Hemi-sensory signs
 Visual field defects
 Seizures (common in neonates)
 Deterioration in level of consciousness (seen in progression of
bleed)
 Headache

140
 Nausea and vomiting

Investigations
 FBC, differential count, ESR, random blood sugar, sickling test,
clotting defects (e.g. protein C & S deficiencies, Antithrombin
III deficiency)
 Clotting profile
 Electrolytes initially, then daily until stable
 Blood, urine and CSF cultures if febrile (remember to withhold
LP if evidence of ↑ICP)
 After stabilization , Neuro-imaging (preferably MRI) and
angiography

MRI brain scan Outline area affected (thrombosis, bleed, abscess,

tumour, etc.
CT scan If MRI unavailable (to exclude haemorrhage)
MRA /MRV Vascular outline to R/O Arteriopathy, Vessel
anomalies and Venous thrombosis
ECG and ECHO cardiac anomaly or arrhythmia

Investigate for a possible thrombophilia even with an obvious

cause like trauma as these two conditions may co-exist.

Management
Ischemic Stroke

 Initial attention to ABCDE

 Consider admission to PICU for 1st 24 hours or until stable
 Monitor vital signs (BP, Pulse, Respiratory rate), input & output,
level of consciousness
 Allow permissive hypertension- Recommended BP goal
between the 50th-95th percentile for age and height, with
permissive hypertension up to 20% above the 95th percentile
o Persistent, significant hypertension should be treated
with caution to lower blood pressure by approximately
25% over 24 hours.
 Nurse head flat for 24 hours (unless signs of raised ICP)

141
 Fluid management- isotonic IVF for at least 24 hours
 Keep nil by mouth
 Maintain normoglycaemia
 Control fever-Maintain normothermia
 O₂ to by face mask if indicated (keep SpO2 greater than 92%)
 Treat acute seizures ( no role for AED's for seizure prophylaxis)
 Manage ↑ICP if present (see management of ICP protocol)
 Control systemic hypertension
 If high index of suspicion for CNS infection give Antibiotics
(Cefotaxime 100 mg/kg q 6 hr or Ceftriaxone 100 mg/kg per
day)
 Other management is dependent on cause, e.g. exchange
transfusion/ blood transfusion in SCD, anticoagulants in
prothrombotic coagulopathy
 Anti viral treatment with Acyclovir has been recommended
for varicella zoster virus vasculopathy with virologic
confirmation of active central nervous system VZV infection

In neonates, treatment for prevention of a 2nd stroke is often not

required because the risk of recurrence is low. In children
however, the recurrence risk is higher and long term therapy with
low dose aspirin is often needed (1-5mg/kg/day not). Aspirin is
recommended for a minimum of two years.

Hyper transfusion therapy and hydroxyurea in patients with SCD

for secondary prevention of Stroke
Refer to specialist for immunosuppressant therapy for vasculitis.

Haemorrhagic stroke

 Stabilize patient ABCDE

 Admit in the PICU
 Monitor for new or worsening neurological symptoms-
extension of haematoma, herniation, hydrocephalous
 Isotonic IVF for at least 24 hours
 Maintain normothemia and normoglycemia
 Maintain normal blood pressure

142
 Manage raised ICP, if present
 Manage acute seizures
 Manage the cause
o Replace clotting factors, platelet transfusion for
thrombocytopenia
o MRI/MRA to look for vascular malformation if possible
o Neurosurgery sometimes can embolize/resect
malformation to prevent future strokes

Rehabilitation
A plan for rehabilitation should be discussed with the parents.
Speech, occupational and physical therapies, psychological
services, special education should be offered.
High risk of future Epilepsy.

Refere nces
Recognition and Treatment of Stroke in Children, Child
Neurology Ad Hoc committee on Stroke in children Rachel U
Sidwell, Mike Thomson, Concise Paediatrics, 1st edition

Kliegman, Behrman, Jenson, Stanton, Nelson Textbook of

Paediatrics, 18th Edition
Robert C Tasker, Robert J McClure, Carlo Acerini, Oxford
Handbook of Paediatrics

Elbers J et al, Paediatrics stroke code: Early management of

child with stroke, Journal of paediatrics, 2015
AHA/ASA Management of stroke in neonates and children,
2019 guidelines

143
GENITOURINARY SYSTEM

URINARY TRACT INFECTION

Definition
UTIs are usually caused when bacteria invade and ascend up
the urinary tract from the urethra and into the bladder. Cystitis, a
lower UTI, occurs when the infection and inflammatory response
are localised to the bladder. Pyelonephritis is an upper UTI in
which the bacteria and subsequent inflammatory response
further ascend to the ureters and kidneys.

Causes
Colonic bacteria are typically the culprits: coliforms Escherichia
coli is the most common bacteria that causes UTIs in all ages,
accounting for 54% to 67% of UTIs in children. Klebsiella (6%-7%),
Proteus (5%-12%), Enterococcus (3%-(%) and Pseudomonas (2%-
6%) are other common causative organisms.

Clinical features
Infants generally present late in the course of infection because
of initial nonspecific signs, such as fever, and the inability to
express symptoms or localize pain.
Older children can usually localise early symptoms of UTI, such as
dysuria or abdominal pain, and therefore present earlier in the
clinical course.
 Fever, Dysuria, Urinary frequency, Suprapubic discomfort,
Flank pain, Costovertebral pain, Abdominal pain.

Urine sampling
 Obtaining urine samples from children who are not toilet
trained involves urethral catheterization, suprapubic
144
 aspiration(SPA), urine collection bag or leaving the child to
void and obtaining a clean catch urine.
 For toilet trained children, a midstream urine sample should be
collected.

Investigations
 Urinalysis
o A positive nitrite test makes UTI very likely, but the test
maybe falsely negative.
o The leukocyte esterase test is an indirect measure of
pyuria.
 Urine microscopy
o A microscopic urinalysis finding of 10 white blood cell
per microliter in uncentrifuged urine specimen is
reported to be a more sensitive indicator.
 Urine culture
o For children who are not toilet trained, only urethral
catheterisation and SPA are considered to be reliable
methods for specimen collection for the purpose of
culture.
o A negative bag culture rules out a UTI but a positive
result is not useful.
Minimum colony counts that are indicative of a urinary tract infection
CFU/ml CFU/L Comments
Clean ≥105 ≥108 Mixed growth is usually
catch(midstream) indicative of contamination.
Sitting a girl backward on the
toilet is a good way to spread
the labia when collecting
midstream urine
In and out catheter ≥5x104 ≥5x107 Mixed growth is usually
specimen indicative of contamination.
Specimens from indwelling
catheters are less reliable
Suprapubic Any Any
aspiration growth growth

145
Treatme nt
 Start empiric treatment with Quinolones but depends on local
susceptible patterns then change according to culture results.
 Oral antibiotics 10 to 14 days or
 IV antibiotics for 3 days ,then 10 days oral antibiotics

Refere nce
Eric Balighian, Micheal Burke: Paediatics in review January
2018,39(1)3-12

HAEMATURIA
Haematuria is defined as the presence of five or more RBCs per
high-power (40x) field in three consecutive fresh, centrifuged
specimens obtained over the span of several weeks.
Confirmation of haematuria is critical. A positive urine dipstick
test may result from myoglobinuria or hemoglobinuria, in which
the urine often is discoloured, but no RBCs are noted on
microscopic evaluation.
Glomerular Versus Extraglomerular Hematuria
Factor Glomerular Extraglomerular
Color Smoky, tea-or cola- Red or pink
colored, red
RBC Morphology Dysmorphic Normal
Casts RBC,WBC None
Clots Absent Present (+/-)
Proteinuria ≥2+

146
 Algorithm for the evaluation of haematuria (adapted from Paediatrics in Review???)

Haematuria

Dipstick (+) Heme

with RBCs on
Gross microscopy Microscopic

(+) Trauma (-) Trauma ???RBCs Dysmorphic RBCs

 Imaging of abdomen/pelvis (+) Pain (-) Pain  Urine Ca/Cr  Evaluate for proteinuria
(Helical CT, RUS, KUB)  R/O ??? stenosis  Electrolytes, BUN, Cr,
 Cystoscopy  Family screening albumin
 C3, C4, ANA
 Streptococcal Abs
 Urine Ca/Cr  Urine Ca/Cr  ANCA
 Urine culture  Evaluate for familial nephritis  CBC
 Imaging for stone  Evaluate for HSP  Evaluate for HSP and
(RUS, CT)  Hb electrophoresis if indicated other vasculitides
147
 History Possible Diagnosis
Lower tract UTI
symptoms(dysuria,urgency,frequency,
suprapubic pain)
Recent illness(pharyngitis,impetigo,viral Postinfectious
illness) glomerulonephritis
Abdominal pain UTI,HSP,Crystalluria/stone
Arthralgias HSP,SLE
Diarrhea(±bloody) HUS
Cough ,hemoptysis Vasculitis
Sickle cell disease Glomerulonephritis,papillary
necrosis
Swimming in streams Schistosomiasis
Physical findings Possible diagnosis
Suprapubic pain UTI
Flank Pain IgAN,stones,renal vein
thrombosis,pyelonephritis
Edema Glomerulonephritis,nephritic
syndrome
Abdominal mass Wills
tumour,hydronephrosis,cystic
kidney disease
Meatal stenosis Infection,trauma
Family History Possible Diagnosis
Sickle cell disease or trait

Diagnostic evaluatio n
The first step involves measurement of blood pressure
 A dipstick urinalysis evaluates for pyuria, proteinuria, heme
positivity, and urinary concentrating defects;
 Microscopy evaluates for white blood cells and clumps, RBC
morphology, crystals and casts.
 Crystalluria can be caused by calcium oxalate, calcium
phosphate, uric acid, or cystine crystals. Hypercalciuria is by
far, the most common cause of crytalluria

148
 Urine culture should be done for suspected UTI.
 Radiographic studies if indictated.
The second stage of evaluation involves a more thorough search
for underlying renal disease, particularly when edema,
hypertension, alterations in urine output, or systemic symptoms
are present.
 Evaluation for sickle cell disease or trait by solubility test and
electrophoresis were available.
 Suspicion of acute PIAGN should prompt ordering of ASO and
other streptococcal antibody titres and a C3 measurement.
 Secondary causes of renal disease warrant checking for
antibodies, complement levels(C3,C4), hepatitis serologies, or
HIV serology.
 A complete blood count is helpful in the setting of petechiae,
bruising, fatigue, abdominal masses, or suspicion of chronic
disease.
 Renal ultrasonography can identify structural abnormality.
 Abdominal radiographs may identify radiopaque stones .

When to refer
 If cause of gross haematuria is unclear refer to next level
 Refer early if patient experiencing symptomatic microscopic
haematuria.
 The patient who has asymptomatic haematuria needs
periodic evaluation every 1 to 2 years to reavaluate for
coexisting symptoms or protienuria and to revisit the family
history with respect to other family members having
haematuria or hearing deficits.
 The child who has persistent asymptomatic haematuria and
concomitant proteinuria needs additional evaluation, often
including a renal biopsy, refer to next level.

Refere nces
Susan F Massengill paediatrics in review October 2008,29(10) 342-
348.

149
NEPHROTIC SYNDROME
Nephrotic syndrome is characterised by:
 Heavy proteinuria > 40 mg/m²/hr or urine protein 3+ or greater
on a dipstick test
 Hypoalbuminaemia (serum albumin < 25g/L)
 Hyperlipidaemia
 Oedema

Causes
 Idiopathic (primary) nephrotic syndrome
 Secondary nephrotic syndrome (secondary to a known cause
e.g. Hepatitis B, malaria syphylis, SLE etc).
 Congenital nephrotic syndrome- occurring in a child less than
three months- it may be primary or secondary.

Clinical features
 Oedema with or without ascites, pleural effusions, and genital
oedema.
 Hypertension and haematuria maybe present.

Investigations
 Blood: FBC/ESR; U&Es; Creatinine; LFTs including serum albumin
; C3/C4 ; ANA, ASOT, anti- Dnase B, RPR, HCV antibodies,
HbSAg, Triglycerides and cholesterol levels.
 Urine: Dipstick urinalysis. Morning urine protein/creatinine ratio
≥ 200mg/mmol or 0.2g/ mmol (morning so as to avoid
orthostatic proteinuria).
 CXR, KUB ultrasound
 If available: Varicella zoster IgG blood levels, tuberculin skin
test.

150
 Renal biopsy only if atypical features at presentation:
macroscopic hematuria, less. than 12 months or older than 12
years, low C3, vasculitic rash, suspected vasculitis.

Treatme nt
Symptomatic treatment
 No added salt diet
 Diuretics- usually Lasix 1mg/kg BD initially- ensure that patient
does not have intravascular volume contraction before
commencing- best to discuss with nephrology first.
 20% albumin 1g/kg given over 4-6 hours with frusemide only if
patient does not respond to Lasix alone. Ensure that patient
does not have renal failure as under these circumstances
albumin can cause pulmonary oedema. Also watch out for
hypertension with this treatment. Further albumin infusions
must only be given on week days and during regular working
hours so that the MO is available to react to any potential
complications.
 Prophylactic antibiotic cover (pen v) as long as patient has
gross oedema including ascites. In penicillin sensitive use
erythromycin
 Daily weight and daily urinalysis
 By and large children with nephrotic syndrome do not need to
be fluid restricted because they have intravascular volume
depletion.
 If antihypertensives are required ensure that the patient has a
normal creatinine and serum potassium before using ACE-
inhibitors. For initial treatment calcium channel blockers such
as amlodipine or nifedipine are ideal- discuss with Nephrology.

Specific treatment
Dose of prednisolone in m2

151
60mg/m2 [ 2mg/kg] in the first 4 weeks and then reduced to
40mg/m2 [ 1.5 mg/kg ] given on alternate days for 4 weeks then
tapered off slowly over another 4-6 weeks.

Definitions
Remission Urine albumin nil or trace (or proteinuria <40 mg/m2 /h) for
3 consecutive days

Relapse Urine albumin 2 + or more for 3 consecutive days, having

been in remission previously
Frequent relapses Two or more relapses in six months of initial response, or
more than four relapses in any twelve months
Steroid Children who relapse whilst on steroids therapy or within
dependence 14 days of discontinuation of steroid therapy
Steroid resistance Absence of remission following at least 28 days of steroid
therapy at a dose of 2 mg/kg per day

Complications
 Increased susceptibility to bacterial infections.
 Spontaneous bacterial peritonitis.
 Hypovolaemia and thromboembolism.
 Complications related to chronic steroid administration
include hypertension, obesity, and linear growth retardation.

Steroid resistant ne phrotic sy ndrome and steroid

depe ndant nephrotic syndro me:
 These patients will require a renal biopsy and introduction of
steroid sparing agents.
 Ideally these patients should be discussed with nephrology
and referred if possible.

Refere nces:
KDIGO glomerulonephritis guidelines
Rees L, Brogan P, Bockenhaeur D, Webb N, “oxford sub-
specialist handbooks in paediatrics: paediatric nephrology”
2nd edition, Oxford University Press, 2012
Avner [editor], Paediatric Nephrology

152
ACUTE GLOMERULONEPHRITIS
Definition
Results from inflammation in the glomerulous. It is a clinical
syndrome that is said to be present when a constellation of four
features are present in a patient.
 Oedema
 Hypertension
 Hematuria
 Renal dysfunction

Causes
Causes of acute glomerulonephritis include post-infectious,
renal, and systemic aetiologies.
Post-infectious aetiologies
 Bacterial: Streptococcus species (i.e. group A beta-
haemolytic), the commonest cause. syphilis, TB, salmonella,
E.coli.
 Viral: Hepatitis B and C, HIV.
 Parasitic: Malaria.
Systemic causes
 Collagen vascular diseases (e.g. SLE)
 Vasculitis- IgA nephropathy, HSP, ANCA associated vasculitis
 Drug-induced (i.e. gold, penicillamine)
 HUS,TTP
 Ventriculo-atrial shunts

Clinical Features
History

 Sudden development of puffiness of the eyelids and facial

oedema in the setting of a post-streptococcal infection.
 The urine may be dark and scanty.
153
 Non-specific symptoms include weakness, fever, abdominal
pain, and malaise.

Physical examination

 Most frequently patients present with a combination of

oedema, hypertension, haematuria and oliguria.
 Look for signs of scabies or furuncles on skin: common in post-
strep
 Other signs depend on the underlying cause e.g. malar rash
and other vasculitic rashes.

Investigations

 Urine microscopy – RBC’s, RBC casts (dysmorphic red blood

cells), proteinuria, leukocytes
 Blood – FBC, U/E and creatinine, ESR
 Cultures – throat swab
 Other (as indicated) - CXR, U/S-renal, ECHO, ASOT,
antinuclear antibody, anti-DNA antibodies.
 Complement levels (C3, C4),ANA, ANCA
 CXR, KUB ultrasound
 +/- renal biopsy

Treatment
 Antibiotic, i.e. penicillin if thought to be post-strep
 Antihypertensive, i.e. diuretics, calcium channel blockers,
beta-blockers.
Important note: Please avoid ACE-inhibitors in this group of
patients as initial antihypertensive choice until blood results for
serum creatinine and potassium are known. And even if
normal cautious use of ACE-I is advised unless one can easily
monitor renal function of the patient.
 Sodium restriction.

154
 Renally adjust all drug doses based on estimated GFR. This
can be calculated using the scwartz formula in children= 40 X
height[ cm]/ serum creatinine (in micromoles/l).
 Fluid restriction may be necessary depending on extent of
renal dysfunction.
 Treat underlying cause.
 If patient progresses to acute renal failure, refer to acute renal
failure protocol.
 As some patients may end up with rapidly progressive
glomerulonephritis early nephrology consultation is highly
encouraged.
 Admit patient initially for investigation.

Post-strep glomerulonephritis

 Commonest cause in our environment.

 Often associated with skin bacterial infection by group A beta
haemolytic streptococcus. Bacterial infection may also be
facilitated by infestation with sarcoptes scabies.
 Usually benign in over 90% of patients but has the potential to
complicate into rapidly progressive glomerulonephritis.
 Expected to have normal C3 by 8 weeks post onset. If not
then patient requires a biopsy.
 If significant proteinuria present after 6 months patient will
need a renal biopsy.
 Patient may continue to have microscopic hematuria for upto
2 years after initial onset.
 Residual hypertension can occur and so patient will need
continued monitoring of blood pressure.

Refere nces
KDIGO glomerulonephritis guidelines
Rees L, Brogan P, Bockenhaeur D, Webb N, “oxford sub-
specialist handbooks in paediatrics: paediatric nephrology”
2nd edition, Oxford University Press, 2012

155
Avner [editor], Paediatric Nephrology

156
ACUTE KIDNEY INJURY

Definition
An acute, potentially reversible, deterioration in kidney function
resulting in failure to maintain normal physiological homeostasis,
characterized by an increase in blood urea and serum
creatinine values, often accompanied by hyperkalaemia,
metabolic acidosis, and hypertension. It may be accompanied
by a urine output < 0.5ml/kg/hour or < 1.0 ml/kg/hour in the
neonate. Note however that some forms of AKI are
accompanied by polyuria e.g. aminoglycoside toxicity.

Pae diat ric RIFLE [ pRIFLE] classification of AKI

 R- risk of renal dysfunction (serum creatininie x 1.5 of baseline).
 I- injury to the kidney (serum creatinine x 2 of baseline).
 F-failure of kidney function (serum creatinine x 3 of baseline).
 L- loss of kidney function.
 E-end-stage renal disease.

Causes
 Pre-renal - Prerenal failure refers to causes resulting from
reduced blood pressure or reduced effective intravascular
volume thus resulting in hypoperfusion of the kidneys. This can
be due to dehydration, haemorrhage, sepsis, cardiac failure,
hypoalbuminaemia (nephrotic syndrome, liver disease).
 Renal (intrinsic) – Renal parenchymal damage, e.g. Acute
glomerulonephritis, Intrarenal vascular disease (Haemolytic-
uremic syndrome, Vasculiditis), Acute interstitial nephritis,
Acute tubular necrosis, Nephrotoxins.
 Post-renal
o Congenital - posterior urethral valves, pelvi-ureteric
junction if bilateral, vesico-ureteric junction obstruction if
bilateral, ureterocoeles, prune belly syndrome, bladder
outlet obstruction, neurogenuc bladder.
o Acquired (rare) – Urolithiasis, Clots, Tumours if both
kidneys involved or if these result in bladder outlet
obstruction.

157
Clinical features
These will reflect the cause and the consequent abnormalities.
 Signs of ARF include signs of fluid overload (hypertension,
oedema) and signs of congestive cardiac failure (CCF), such
as hepatomegaly, gallop rhythm, and pulmonary oedema.
 Signs of intravascular volume depletion include tachycardia,
hypotension, decreased skin turgor, dry mucous membranes,
and changes in sensorium.
 Although oliguria is a criterion used to diagnose and stage
acute renal failure (ARF), ARF can be present without oliguria,
especially in patients with nephrotoxic kidney injury, interstitial
nephritis, or perinatal asphyxia. Oliguria may be defined as
urine output less than 0.5 ml/kg/h in children and < 1mlkg/hr in
neonates.
 Rash and arthritis(e.g. SLE and Henoch-Schonlein purpura
nephritis), flank masses (renal vein thrombosis, tumours, cystic
diseases, urinary tract obstruction)

Investigations
 Dipstick urinalysis: leucocytes (UTI), Proteinuria & Haematuria
(Glomerulonephritis, nephrotic/nephritic syndrome), specific
gravity.
 Urine microscopy: red blood cell casts (dysmorphic red blood
cells), granular casts, and red blood cells, findings seen in
glomerulonephritis.
 Urine: sodium and urine osmolality.
 Serum bicarbonate: if not done by lab routinely can be
obtained from blood gas strip.
 Electrolytes: K+, Na+, Ca++, Phosphate, Uric acid and urea,
creatinine.
 Full blood count: peripheral smear as indicated.
 Other blood tests as dictated by underlying cause.
 CXR: pulmonary congestion (fluid overload).
 KUB ultrasound (urinary tract obstruction).
158
 KUB x-ray
 +/-Renal biopsy: ultimately if cause unclear or suspected
rapidly progressive glomerulonephritis.

Treatme nt
 Pass urinary catheter e.g. in neonates with suspected posterior
valves, or for urine output monitoring in non-ambulatory
children/adolescents during ARF. If size 6 Fr foley catheter is
unavailable one can easily use a size 6 Fr feeding tube [NGT]
to catheterise the bladder. This can be held in place by
strapping to the thigh of the infant so that it does not fall out.
 NOTE: in cases of PUV please note that the patient may
develop post-obstruction polyuria once a urinary catheter is
inserted. If not properly managed the infant may become
severely fluid depleted and develop a pre-renal AKI or even
go into shock and die. Thus monitor urine output and 6 hourly
weight changes meticulosly. Ideally use hypotonic solutions
such as half strength darrows in older infants and quarter
strength darrows in the newborn. This is because the post-
obstructive kidney often has a poor concentrating ability.

 Fluids – if hypovolaemic will require volume expansion with

isotonic saline at 20 ml/kg over 30 min. This may be repeated
one or two more times at most depending on the response.
 Those with relatively normal intravascular volume – give
insensible losses at 400 ml/m2/day (30ml/kg/day) plus urine
output for the previous 24 hours & extra renal losses as
appropriate.
 Daily monitor urine & stool output, fluid intake, body weight,
blood chemistries.
 Renally adjust all drug doses. Use the Schwartz formula to
calculate the estimated GFR
 Involve your nearest paediatric nephrology unit earlier rather
later in the management of all paediatric AKI patients as per
KDIGO AKI guidelines

159
Complications
 Hyperkalaemia > 6 mmol/l (indicated by the following ECG
changes: peaked T-waves, widening QRS complex, ST
segment depression, ventricular arrhythmias and cardiac
arrest).
 Eliminate exogenous potassium (IVF, diet) including drugs
sources, oral or enema K+-binding resin (Na polystyrene
sulfonate [ kayexalate]) at 1g/kg single dose.
 If K+ >7 mmol/l, + ECG changes, give the following:
 Nebulised salbutamol 2.5 mg if weight < 25kg or 5 mg if weight
>25 kg or IV salbutamol 4µg/kg in 10 ml of water over 10 min
 10% calcium gluconate (cardio-protective) at 0.5ml/kg IV
over 10 min
 8.4% sodium bicarbonate 1-2 mmol/kg IV over 5-10 min
 Regular insulin 0.1 u/kg with 50% glucose 1 ml/kg over 1hr
 Lasix at 1mg/kg BD may also be used to drive out potassium
 The above are only temporizing measures. Please discuss with
nephrology for arrangement of dialysis ASAP.

Hypocalcaemia

Calcium carbonate orally 45 to 65 mg/kg per day. If tetany, IV

10% calcium gluconate 0.5 to1 ml/kg (up to 10 ml) if patient is
symptomatic, with low phosphorus diet or phosphorus binders
(calcium carbonate given with meals).

Hyponatraemia (dilutional usually)

Treat if < 120 mmol/l or symptomatic (seizures, lethargy). Correct

to 125 mmol/l using the following formula:
Required NaCl (mmol) = 0.6×weight×deficit (125 mmol/l – serum
Na) given over 4-6 hours
 One would need to use 3% hypertonic saline if not available
discuss with pharmacist how NaCl tablets or table salt can be
given orally in-lieu of hypertonic saline

160
Metabolic acidosis

Mild acidosis is common and requires not treatment. However,

severe acidosis (pH = 7.15, serum bicarbonate ˂ 8 mmol/l) should
be corrected partially with IV sodium bicarbonate to pH = 7.20,
serum HCO3- = 12 mmol/l, then the remainder corrected by oral
sodium bicarbonate. Rapid metabolic acidosis correction with IV
NaHCO3 may reduce ionised serum calcium leading to tetany!
Preferably correct metabolic acidosis by instituting dialysis
Hypertension and Anaemia treat as per appropriate protocol.

Nutrition
Nutritional requirements should be addressed accordingly in
consultation with the dietician.

Renal replacement ther apy

Dialysis (peritoneal dialysis or haemodialysis)
Dialysis indications:

 Fluid overload/hypertension
 Severe acidosis not responding to medical management
 Persistent hyperkalaemia or other above uncontrollable
electrolyte imbalance (iv). Symptomatic uraemia or urea >
35.7 - 53.6 mmol/l
 Dialyzable toxin
 Established anuria

Refere nces
KDIGO AKI guidelines
Rees L, Brogan P, Bockenhaeur D, Webb N, “oxford sub-
specialist handbooks in paediatrics: paediatric nephrology”
2nd edition, Oxford University Press, 2012
Avner [editor], Paediatric Nephrology

161
162
GASTROINTESTINAL SYSTEM

ACUTE DIARRHOEAL DISEASE

Definition
Passage of three or more loose/watery stool or one voluminous
loose/watery stool per day.

Causes
 Infectious
o Bacterial infections: Commonly, Vibrio cholerae,
Salmonella species, Shigella, and Escherichia coli (E.
coli), Campylobacter pylori
o Viral infections: rotavirus, novovirus, adenovirus, Norwalk
virus, astrovirus
o Parasites: Giardia lamblia, Entamoeba histolytica,
Cryptosporidium
N.B. Diarrhoea may be watery or bloody (dysentery)
o Systemic infections associated with diarrhoea include:
 influenza, measles, fever, HIV, malaria,
pneumonia, urinary tract infection, meningitis,
and sepsis.
 Non-infectious causes
o Food poisoning
o Drugs: Antibiotics, anti-hypertensive, cancer drugs, and
antacids containing magnesium
o Intestinal diseases: Inflammatory bowel disease and
coeliac disease
o Food intolerance: Lactose
o Food allergy: Cow’s milk, Soya
NOTE: Some of the above causes may lead to
persistent/chronic diarrhoea

Clinical features:
 Watery or loose stools ± bloody stools
 Abdominal cramps
163
 Tenesmus
 Urgency
 Abdominal pain
 May be associated with vomiting and fever, poor appetite
 Dehydration

Clinical assessment
Divided into four components to guide clinical management:
 Classification of the type of diarrhoeal illness
 Assessment of hydration status
 Assessment of nutritional status
 Assessment of co-morbid conditions

Signs of dehydratio n
 Dry mucous membranes
 Rapid thready pulse, low blood pressure, capillary refill > 2sec
 No tears when crying
 No wet diapers for 3 hours or more
 Sunken eyes/anterior fontanelle
 High /low temperature
 Listlessness or irritability
 Reduced skin turgor

Classification of levels of dehydration; using a combination of

two to three physical signs reliably predict dehydration.

No dehydration Some Severe dehydration

dehydration
General Well, alert Restless, irritable Lethargic or
condition unconscious
Eyes Normal Sunken Very sunken

Thirst Drinks normally, not Thirsty, drinks Drinks poorly, or not

thirsty eagerly able to drink at all
Skin pinch Goes back quickly Goes back slowly Goes back very
(< 2sec) slowly (>2sec)
Treatment PLAN A PLAN B PLAN C

164
Investigations
 FBC, ESR
 Stool m/c/s for bloody and PDD
 U&E, Creatinine
 Abdominal x-ray
 Barium enema/meal
NOTE: Other investigations will depend on the
underlying/systemic conditions identified such as RDT/MPS,

Principles o f manage ment

 Fluid replacement
 Zinc supplements
 Continued feeding
 Antibiotics

Fluid management
Intravenous fluids are required in the following cases (in all others,
ORS should be preferred):

 Resuscitation from shock

 Dehydration with severe acidosis and prolonged capillary refill
time
 Severe abdominal distension and ileus
 An altered level of consciousness
 Resistant vomiting despite appropriate oral fluid administration
 Deterioration or lack of improvement after 4 hours of
adequate oral fluids:
o Check vital signs
o Assess and grade hydration status
o If in shock, refer to protocol on shock
o Depending on level dehydration, give fluids as outlined
below

165
PLAN C– Se vere dehy dratio n:
Rapid intravenous rehydration, Give 100 ml/kg RL or ½ strength
Darrow’s with 5-10% dextrose:

Age First give 30 ml/kg in Then give 70 ml/kg in

Infants 1 hour * 5 hours

Older children 30 min * 2½ hours

Reassess patient every 1-2 hours. If hydration is not improving,

give the IV drip more rapidly.

 After completion of IV fluids, reassess the patient and choose

the appropriate treatment Plan (A, B or C).
 Repeat Plan C once if no improvement.
 If IV therapy is not available, then ORS by nasogastric tube or
orally at 20 ml/kg/hour for 6 hours (total of 120ml/kg) should
be given. If abdomen becomes distended or the child vomits
repeatedly, then ORS should be given more slowly.
NOTE: Dehydration may be hypotonic, isotonic or hypertonic.
Hypertonic dehydration is common in infants fed on cow’s
milk.

PLAN B – So me de hydration:
75mls of ORS x patient’s weight (kg) to be given in 4 hours
The approximate amount of ORS required (in ml) can be
calculated by multiplying the child’s weight (in kg) by 75, to be
given in 4 hours.
 After 4 hours, reassess the child and decide what treatment to
be given next as per level of dehydration.
 Children who continue to have some dehydration even after
4 hours should receive ORS by nasogastric tube or ½ strength
Darrow’s intravenously (75 ml/kg in 4 hours).

166
 In case of Resistant vomiting despite appropriate oral fluid
administration, IV fluids may be used.
o Avoid Promethazine (Phenergan)
o Ondansetron may be used up to two doses
 If abdominal distension occurs, oral rehydration should be
withheld and only IV rehydration should be given.

PLAN A – No dehydration:
Amount of ORS to be given per loose stool dependent on
specific age as listed below:
Age (years) <2 2-5 Older children
ORS (mls) 50-100 100-200 As much as they
want

Zinc supplementation
Give zinc supplement for 10 to 14 days
 Infants below 6months of age 10mg daily
 Children 6months and above 20mg daily

Continue d feeding
Nutritional Status:
 Children presenting with diarrhoea should be assessed for
malnutrition according to WHO standards.
 Children with acute diarrhoea and malnutrition are at
increased risk for developing fluid overload and heart failure
during rehydration.
 The risk of serious bacterial infection is also increased.
 Such children require an individualized approach to
rehydration and nutritional care.
NOTE: Diarrhoea is a major risk factor for malnutrition which is
associated with high mortality and deficits in physical and
cognitive development.

167
Give appropriate feeds. Avoid juices and carbonated drinks

Drugs
 Antibiotics are not indicated for most children with acute
watery diarrhoea; dysentery and suspected cholera are
important exceptions.
 Children with acute diarrhoea should NOT receive antimotility
agents or antiemetics.
NOTE: Antimotility agents (loperamide, diphenoxylate-
atropine, and tincture of opium) prolong some bacterial
infections and may cause fatal paralytic ileus in children

Refere nces
Outhall D, Coulter B, Ronald C, International Child Health
Care, A practical manual for hospitals worldwide. 1 st Edition,
Book Power, in: BMJ Publishing Group, 2003.
K Reddy; M E Patrick - Management of acute diarrhoeal
disease at Edendale Hospital, in: S. Afr. j. child
health vol.10 n.4;10(3):215-220. Cape Town Dec. 2016
WHO-Hospital care for children, 2013 edition.
Ryan ET, Dhar U, Khan WA, et al. Am J Trop Med Hyg 2000;

168
PERSISTENT DIARRHOEA (PD)
Passage of 3 or more watery stools within 24hrs lasting for more
than 14 days.
 Though PD accounts for 2-20% of total diarrhoea cases, it
accounts for 23-62% of all diarrhoea related deaths.
 Dehydration, malnutrition and infections are major
contributors to PD morbidity.
 The many causes of PD can be divided into four principle
pathophysiologic mechanisms: osmotic, secretory, dysmotility
associated, and inflammatory.

Clinical features
 Liquid stools often passed after eating, may be explosive
 Occasionally stool may contain visible blood
 Weight loss often evident and signs of malnutrition often
present
 Signs of dehydrations
 Features of Extra-intestinal infections: e.g. Pneumonia, UTI

Causes
Major Clinical Features Laboratory and Imaging
Cause (In addition to PD) Findings

Infectious (e.g. E. coli, Possible blood and/or Positive stool culture,

Cryptosporidium. mucus in stool ova and parasite
Giardia, Salmonella, E. Possible fever and/or examination,
histolytica) abdominal pain
Lactose malabsorption Abdominal discomfort, Elevated breath
bloating, flatulence hydrogen
concentration
after lactose ingestion
Immunodeficiency Recurrent infections Abnormal
state Young age, typically in immunoglobulins (eg,
(various diseases) infancy low IgG, low IgA, high
IgM)
Lymphopenia
Low antigen titers to
previous immunizations
Food Allergy Most commonly in May have
response to cow or hypoalbuminemia and
soy milk anemia

169
 Electrolyte abnormalities
from diarrhea/ vomiting
Serum IgE may be
elevated
Hirschsprung disease Delayed passage of Abnormal barium
meconium Abnormal enema
barium enema Absent ganglion cells on
Distended abdomen rectal biopsy
Explosive stool with rectal
examination
Toddlers diarrhoea Usually thriving toddler. Normal laboratory and
Sweetened juices usually imaging results
the cause
IBS Alternating constipation Normal laboratory and
with diarrhea imaging results
Abdominal pain relieved
by defecation
Typically diagnosed in
adolescence
or later
Celiac disease FTT, abdominal Elevated anti-TTG IgA,
distention, vomiting antiendomysial IgA
Typically 9-24mo of age Antibodies
IBD Bloody stool Elevated ESR & fecal
Stooling urgency, calprotectin
abdominal pain, thrombocytosis
Fatigue. Weight loss. Iron-deficiency anemia
Arthritis Hypoalbuminemia

Treatme nt Objectives
Treatment of PD consists of:
 Appropriate fluids to prevent and treat dehydration (refer to
table xxx)
 A nutritious diet to promote weight gain. BEWARE of foods
worsen diarrhea
 If SAM is present, treat according to SAM protocol (refer to
section on SAM)
 Supplementary vitamins and minerals, including zinc for 10-14
days
 Antimicrobials to treat diagnosed infections

170
Refere nce
B. Umamaheswari, Niranjan Biswal, B. Adhisivam, S.C. Parija1
and S. Srinivasan. Persistent Diarrhea: Risk Factors and
Outcome. Indian J Pediatr. 2010; 77 (8): 885-888.
Zella G, Israel EJ. Chronic diarrhea in children. Pediatrics in
Review. 2012: vol 33 no 5

171
ACUTE UPPER GI BLEEDING (OLD)

Definition
Upper gastrointestinal (GI) bleeding refers to haemorrhage from
any level above the ligament of Treitz.

Causes :
Age Group Cause
Neonates  Haemorrhagic disease of the newborn
 Swallowed maternal blood
 Stress ulceration
 Coagulopathy
Infants (1 month – 1 year)  Oesophagitis
 Gastric ulceration
Infants (1 -2 years)  Peptic ulcer disease
 Gastritis
Children older than 2 years  Oesophageal varices
 Gastric varices
Adolescents  Duodenal ulcers

Clinical features
Presentation of bleeding depends on the amount and location
of haemorrhage.
 Haematemesis
 Coffee ground vomiting
 Melaena
 Haematochezia- if the haemorrhage is severe
 May also present with complications of anaemia/shock

Investigations
 FBC, ESR
 U/E’s, Creatinine, LFTs
 Barium swallow/meal

172
 Clotting profile
 Endoscopy

Treatme nt
 ABCDE
 Brief history as to possible cause of the bleeding
 Consider gastric wash out
 Consider antidote for bleeding due to poisoning
o Iron – Desferrioxamine
o Warfarin – Vitamin K
 Monitor vital signs
 Replacement of volume with intravenous solutions and blood
products if required
 Endoscopy (electrocautery, clipping or banding)
 Pharmacotherapy including the following:
o Proton pump inhibitors (PPIs) – Omeprazole IV
o H2 receptor inhibitors - Cimetidine/ranitidine IV
o Octreotide is a somatostatin analog believed to shunt
blood away from the splanchnic circulation
o Terlipressin is a vasopressin analog used for variceal
upper GI haemorrhage
o Propranolol

Refere nces
Kliegman R, Berhman R et al; Nelson Textbook of Pediatrics;
Saunders Elsevier; 18th ed, 2007

McIntosh N, Helms J. P, Smyth L. R, Logan S. Forfar & Arneil’s

textbook of paediatrics, 7th edition, Edinburgh, Churchill
Livingstone, 2008

173
FUNCTIONAL CONSTIPATION
This is amongst the commonest conditions associated with
chronic or recurrent abdominal pain in children. Usually during
weaning, toilet training and beginning of schooling as well as
during stressful events. History and examination is usually
sufficient to make a diagnosis. A plain abdominal X-ray may
help make a diagnosis.
Features last 2 weeks or more and may include:
 Non-specific chronic/recurrent abdominal pain.
 ≤2 defecations per week.
 At least one episode/week of incontinence after the
acquisition of toileting skills
 History of retentive posturing or excessive stool retention
 History of painful or hard bowel movements
 Soiling of underwear
 History of large diameter stools

Accompanying symptoms may include:

o irritability,
o decreased appetite, and/or
o early satiety.
NOTE: Accompanying symptoms disappear immediately
following passage of a large stool.
Review feeding practices (formula, fibre/water intake).

Red Flags
The presence of the following signs warrants further investigation
or referral.
 Fever
 Blood in stool
 Weight loss
 Vomiting
 Anemia

174
Differe ntial diagnosis
 UTI
 PUD
 Hypothyroidism
 Rarely: IBS and IBD
NOTES: Neonates/Infants: Obtain good history to rule out
congenital causes of constipation (Hirshsprung’s disease,
spine abnormalities, metabolic/endocrine, etc).

Treatme nt
 Parental/patient education and behaviour interventions are
paramount (toilet training coupled with a reward system).
 Diet modification (increase fluids, fibre and fruits).
 Disimpaction with either polyethelene glycol (PEG) or
lactulose +/- enema.
 Maintenance therapy with either polyethelene glycol,
lactulose or liquid
 paraffin. Others include Magnesium hydroxide, magnesium
citrate, senna and sorbitol.
 Counsel parents on need for prolonged treatment duration
(usually months) to avoid recurrence and overcome fear of
pain during defecation in the child.

Refere nces
Rome III Diagnostic Criteria for Functional Gastrointestinal
Disorders.2006
Borowitz SM, Windle ML, Cuffari C. Liacouras CA. Pediatric
constipation. Medscape. Updated May, 2019.
Nurko S and Zimmerman LA. Evaluation and treatment of
constipation in children and adolescents. American
Academy of Family Physicians. 2014;90(2):80-90

175
176
ACUTE HEPATIC FAILURE (OLD)

Definition
Fulminant hepatic failure (FHF) is usually defined as the severe
impairment of hepatic functions in the absence of preexisting
liver disease.

Causes
 Infective
 Viral - Hepatitis A, B, C and D, HIV, Parvovirus, Herpesvirus,
Enterovirus, Adenovirus, Varicella, Echovirus, CMV
 Drugs - Paracetamol, antituberculous drugs, carbamazepine,
sodium valproate, halothane
 Toxins - Mushroom, particularly amanita phalloides, herbs and
traditional medicines
 Infiltrative - Leukaemias, lymphomas
 Metabolic - Wilson’s, galactosemia, tyrosinaemia

Clinical Features
The child may present within hours or weeks with
 Protracted vomiting
 Jaundice
 Tender hepatomegaly
 Coagulopathy (bruising, petechiae, bleeding)
 Hypoglycaemia
 Electrolyte disturbance
 Encephalopathy (early signs of encephalopathy include
alternate periods of irritability, confusion and drowsiness. Older
children may be aggressive or show unusual behaviour)

Investigations
 Liver Function tests:
o Prothrombin time (PT) or International normalised ratio
(INR)
o Serum albumin
177
 o Transaminases
o Bilirubin total and direct
o Alkaline Phosphatase
 Blood Glucose levels
 Full Blood Count
 Urea, creatinine and electrolytes
 CRP/ESR
 Imaging
 Investigate for underlying cause

Treatme nt
 ABCDE
 Oxygen by nasal cannulae or face mask
 Vitamin K, stat dose IV or IM (300 micrograms/kg for age 1
month to 12 years and 10 mg if >12 years to attempt
correction of prolonged clotting time
 If frank bleeding (GI or other), fresh frozen plasma at 10 ml/kg
IV
 Maintain blood glucose between 4 and 9 mmol/litre using 2/3
of maintenance fluid volume consisting of 10% dextrose IV or
orally
 Strictly monitor urine output and fluid balance, aim for a urine
output of not less than 0.5 ml/kg/hr
 Correct hypokalaemia if present as it can worsen
encephalopathy
 Broad spectrum antibiotics for example cephalosporin to treat
sepsis
 Systemic fungal infection may require IV amphotericin or oral
fluconazole
 Maintain normothermia by environmental measures, do not
give paracetamol
 Lactulose 5-10 mls 2-3 times daily to produce two to four soft
and acid stools per day, to be omitted if diarrhoea occurs
 Neomycin 20-30 mg/kg/day 6 hrly orally, maximum dose 2
gm/day

178
Refere nces
Kliegman R, Berhman R et al; Nelson Textbook of Pediatrics;
Saunders Elsevier; 18th ed, 2007
McIntosh N, Helms J. P, Smyth L. R, Logan S. Forfar & Arneil’s
textbook of paediatrics, 7th edition, Edinburgh, Churchill
Livingstone, 2008

179
INTESTINAL OBSTRUCTION
This is the most common condition requiring emergency surgery
in infants and children. Most causes result from complications of
congenital anomalies or from inflammatory conditions that
affect the bowel.

Causes
A. Small bowel obstruction
o Duodenal stenosis or atresia– One third of patients with
Down’s syndrome and it is also associated with other
congenital malformations
o Atresia or stenosis of the jejunum or ileum
o Malrotation with volvulus
o Meconium ileus
o Meconium plug

B. Large bowel obstruction

o Mainly caused by peristaltic dysfunction as in
Hirschsprung’s disease and congenital anomalies (rectal
atresia).
C. Mixed (small or large bowel)
o Inflammatory lesions like tuberculosis and Crohn’s
disease
o Worm infestation
D. Extrinsic causes
o Incarcerated hernia
o Vascular bands
o Intussusception.

Symptoms and Signs

 Vomiting which progresses to become bowel stained
 Cramping abdominal pain with anorexia
 Constipation

180
 Abdominal distension which is greater the more distal the
obstruction
 Tachycardia and signs of dehydration
 Abdominal tenderness
 Hyperactive or absent bowel sounds

Investigations
 Full blood count
 Urea, creatinine and electrolytes
 Group and save
 Abdominal x-rays, supine and erect
 Chest x-ray
 Barium meal is needed for bilious vomiting and for incomplete
obstruction
 Barium enema may be required for distal obstruction

Treatme nt
The goal of treatment is to relieve the obstruction before
ischemic bowel injury occurs. The patient should be resuscitated
in the following way:
 Keep NPO
 Give Oxygen if saturations ≤ 92 %
 IV access and collect blood for investigations
 Rehydrate according to the level of dehydration
 Give maintenance fluids if patient is not dehydrated
 If patient is hypokalaemic, add potassium chloride to fluids
 Nasogastric tube for gastric decompression
 Give IV 10% dextrose if patient is hypoglycemic
 Fluid input and Output balance chart
 Broad spectrum antibiotics: Triple antibiotic therapy:
o Crystalline Penicillin, 50 000IU/kg/dose QID IV
o Gentamycin, 7.5mg/kg/day OD IV
o Metronidazole, 7,5mg/kg/dose TDS IV
 Give adequate analgesia

181
Once the patient is adequately resuscitated and fluid and
electrolyte imbalances corrected, laparotomy is performed and
the cause treated.

Refere nces.
Kliegman R, Stanton B et al; Nelson Textbook of Paediatrics
20th Edition, Elsevier, 2016
McIntosh N, Helms J. P, Smyth L. R, Logan S. Forfar & Arneil’s
textbook of paediatrics, 7th edition, Edinburgh, Churchill
Livingstone, 2008

182
INTUSSUSCEPTION

Definition
Intussusception refers to the invagination (telescoping) of a part
of the intestine into itself. It is the most common abdominal
emergency in early childhood, particularly in children younger
than two years of age. Ileocolic intussusception accounts for 90%
of all cases.

Clinical Manifestat ions

 Typically presents between 6 and 36 months
 Intermittent, severe, crampy, progressive abdominal pain of
sudden onset
 Inconsolable crying and drawing up of legs to the abdomen
 Relative calm between paroxysms of pain
 Vomiting, non-bilious initially but later bilious as condition
progresses
 Stool with red blood and mucus, currant jelly stool
 Palpable sausage shaped mass in the right upper quadrant
 Progressive weakness and lethargy
 Shock like state with fever and peritonitis may develop

Diagnosis
 Ultrasonography which shows the classic “target sign”
 Plain abdominal X-ray to rule our perforation

Treatme nt
 Refer to intestinal obstruction for notes on how to stabilize the
patient.
 Reduction of an acute intussusception is an emergency
procedure and should be performed immediately after
diagnosis in preparation for possible surgery.
 Non-operative reduction in patients with no evidence of
bowel perforation by hydrostatic or pneumatic pressure.
 Surgical intervention in patients who are acutely ill or have
evidence of perforation and where radiographic facilities and

183
 expertise to perform non-operative reduction are not readily
available.

Refere nces
Kliegman R, Stanton B et al; Nelson Textbook of Paediatrics
20th Edition, Elsevier, 2016

Nghia, JV. Thomas TS. Intussusception in Children. In:

UpToDate, Jonathan/Singer (Ed), UpToDate, Waltham MA,
2019

184
INFANTILE COLIC
It is a behavioural syndrome in neonates and infants that is
characterized by excessive, paroxysmal crying. For clinical
purposes it is defined broadly as crying for no apparent reason
that lasts for ≥3 hours per day and occurs on ≥3 days per week in
an otherwise healthy infant <3 months of age.
It is a poorly understood phenomenon with no difference in
incidence between males and females, breastfed and formula-
fed or full term and preterm infants

Clinical Manifestat ions

 It is paroxysmal and occurs mostly in evening hours
 The crying is louder, higher and more variable in pitch and
more turbulent than non- colicky crying
 Associated tensing of the abdomen with drawing up of the
legs
 Clenching of the fingers and stiffening of the arms
 Arching of the back
 Difficult to console with relief at times after passage of flatus or
stool

Diagnosis
 The diagnosis is confirmed in retrospect after it has run its
characteristic course
 Other causes of prolonged crying need to be excluded:
o Hunger/Inadequate feeding
o Diaper pin poking the skin/Diaper rash
o Trauma (abusive/non-abusive)
o Corneal abrasion or foreign body
o Otitis media
o Oral candidiasis
o UTI
o Meningitis

Treatme nt

185
 The goal of management is to help parents cope with the
child’s symptoms and prevent long term sequelae in the
parent-child relationship
 Drug treatment generally has no place in the management of
colic
 Parental support is the mainstay of management
 Parental education and support should include:
o Reassurance that it is common and usually resolves
spontaneously by three to four months of age
o Reassurance that the infant is not sick.
o Education that colic is not caused by something they
are doing or not doing
o Providing tips and techniques to soothe baby
o Reassurance that the infant is difficult to soothe. This
prevents the parents feeling like they have failed
o Encourage parents to access additional caretakers
when they are overly tired or stressed
 Parents should experiment with several soothing techniques
and continue with those that are helpful. Some techniques
include:
o Using a pacifier
o Rocking the infant
o Changing the scenery
o Providing a warm bath
o Rubbing the infant’s abdomen

Refere nces
Deshpande, GP. (2017). Colic. Medscape
Turner TL, Palamountain S. Infantile Colic: Management and
Outcome. In: UpToDate, Augustyn M (Ed), UpToDate,
Waltham MA, 2014.

Gail M Cohen, Laurie W Albertine. In Brief. Pediatrics in

Review July 2012, 33 (7) 332-333

186
187
GASTROESOPHAGEAL REFLUX DISEASE (GERD)
Gastroesophageal reflux disease (GERD) is a condition that
develops when reflux of stomach contents causes troublesome
symptoms and/or complications.

Diagnosis
Can be based on Signs and Symptoms

Infants Older Children and Adolescents

Feeding resistance Abdominal pain
Recurrent vomiting Heartburn
Failure to thrive Recurrent vomiting
Fussiness/irritability Dysphagia
Apnea/choking episodes Chronic cough/wheezing
Opisthotonic posturing Hoarseness
Poor weight gain
Excessive crying
Disturbed sleep

Diagnostic Evaluation of Pediatric GERD

 History and physical examination
 Upper gastrointestinal series
 Esophageal pH monitoring
 Esophagoscopy with biopsy ( Rule out Eosinophilic
esophagitis)

Goals fo r treatment of GERD are to:

 Relieve symptoms
 Heal esophagitis if present
 Maintain remission of symptoms, and
 Manage or prevent complications.
Treatment options to achieve these goals include dietary or
behavioral modifications, pharmacologic intervention, and
surgical therapy.

188
Lifestyle C hanges in t he Manage ment of Pe diatric
GERD sho uld be the initial form of manage me nt
before me dical.
Infants Older Children and Adolescents
Thickened feedings Weight reduction if overweight
Smaller, more frequent feedings Dietary modification
Anti-reflux positioning after feedings Smoking cessation
Avoid passive tobacco smoke
exposure

Drug treatment
Three classes of drugs can be used in infants and children who
do not respond to feeding modification and positioning:
 Histamine-2 (H2) blockers
 Proton pump inhibitors (PPI)
 Promotility drugs
Typically, treatment is begun with an H2 blocker such as
ranitidine. If the infant responds, the drug is continued for several
months and then tapered and stopped (if possible). If infants fail
to respond to H2 blockers, a PPI such as Omeprazole can be
considered. PPIs are more effective at suppressing gastric acid
than are H2 blockers. For infants with GERD and an acute
symptom such as irritability, a liquid antacid can be used.
Infants who have gastroparesis may benefit from a promotility
drug in addition to acid-suppressive therapy. Erythromycin is one
of the most commonly used promotility drugs for this situation.

Medication Dose Frequency

H2RAs
Cimetidine 40 mg/kg/day Thrice daily or 4 times daily
Famotidine 1 mg/kg/day Twice daily
Ranitidine 5–10 mg/kg/day Twice daily or thrice daily
PPIs

189
 Medication Dose Frequency
Lansoprazole 0.4–2.8 mg/kg/day Once daily
Omeprazole 0.7–3.3 mg/kg/day Once daily
PROMOTILITY DRUGS
Erythromycin 0.15mg/kg Four times daily
Metoclopromide – 0.2mg/kg Four times daily
(max 10mg/dose)
Domperidone – 0.2mg/kg Four times daily
(max 10mg/dose)

Surgery:

Patients with severe or life-threatening complications of reflux

that are unresponsive to medical therapy can be considered for
surgical therapy. The main type of antireflux surgery is
fundoplication

Refere nces
Nelson SP, Chen EH, Syniar GM, Christoffel KK. Prevalence of
symptoms of gastroesophageal reflux during infancy: a
pediatric practice-based survey. Pediatric Practice
Research Group. Arch Pediatr Adolesc Med. 1997; 151: 569-
572.

Nelson’s Textbook of Pediatrics, 20th Edition

190
PROLONGED JAUNDICE

Definition
Prolonged neonatal jaundice is defined as a jaundice lasting
more than 14 days of life in the full-term infants. Etiologically it is
helpful to distinguish jaundice related to unconjugated (indirect)
or conjugated (direct) hyperbilirubinemia.

Causes
A prolonged unconjugated hyperbilirubinemia may be related
to breastfeeding or to some pathological conditions as
hemolytic diseases (due to Rh or AB0 incompatibility, or G6PD
deficiency), congenital hypothyroidism, urinary infection, Crigler-
Najjar or Gilbert syndromes.
Conjugated hyperbilirubinemia (cholestasis jaundice) is never
physiologic. It affects 1/2500 live births and it should be
suspected in all jaundiced infants with light stools and dark urine.

191
192
193
Refere nces
Nelson’ Textbook of Pediatrics 20th edition
American Academy of Pediatrics in Review

194
PEPTIC ULCER DISEASE

Definition
Peptic ulcer disease (PUD), the end result of inflammation
caused by an imbalance between cytoprotective and cytotoxic
factors in the stomach and duodenum, manifests with varying
degrees of gastritis or frank ulceration.
The pathogenesis of peptic ulcer disease is multifactorial, but the
final common pathway for the development of ulcers is the
action of acid and pepsin-laden contents of the stomach on the
gastric and duodenal mucosa and the inability of mucosal
defense mechanisms to allay those effects.
Gastric ulcers are generally located on the lesser curvature of
the stomach, and 90% of duodenal ulcers are found in the
duodenal bulb.
The common causes of peptic ulcers include H pylori,
medication use, and stress-related gastric injury. Less common
causes include ingestion of corrosive substances, hypersecretory
states (Zollinger-Ellison syndrome), IBD, systemic mastocytosis,
chronic renal failure, and hyperparathyroidism.

Symptoms and signs

Vary with the age of the patient.
 Hematemesis or melena is reported in up to half of the
patients with peptic ulcer disease.
 Epigastric pain and nausea, especially in school going
children.
 Dyspepsia, epigastric abdominal pain or fullness, is seen in
older children.
 Infants and younger children usually present with feeding
difficulty, vomiting, crying episodes, hematemesis, or melena.
 In the neonatal period, gastric perforation can be the initial
presentation.

Examinatio n
Includes weight-for-height and/or BMI should be calculated,
oropharynx for caries and eroded enamel, Pale conjunctiva,
195
tachycardia, or flow murmur, Halitosis, wheezing, areas of
tenderness on abdomen

Diagnosis
 Esophagogastroduodenoscopy is the method of choice.
 Safely performed in all ages by experienced pediatric
gastroenterologists. Endoscopy allows the direct visualization
of esophagus, stomach, and duodenum.
 Biopsy specimens may be obtained from the stomach for
histopathology, culture or rapid urease testing for H. pylori.
 Endoscopy also provides the opportunity for hemostatic
therapy including injection and the use of a heater probe or
electrocoagulation if necessary.
 Other investigations include faecal-H. pylori antigen test and
urea breath test.
 *Blood-H. pylori antigen test and abdominal ultrasound are
less informative hence a diagnosis should not be based solely
on these.

Treatme nt
PUD treatment includes eradication of H. pylori and gastric acid
suppression using triple therapy comprising of 2 antibiotics and a
proton pump inhibitor (PPI). Examples of triple therapy regimens
are shown in the table xx
Medication Dosage Duration
Omeprazole 1mg/Kg/day in 2 divided doses 1 month
Amoxillin 50mg/Kg/day in 2 divided doses 14 days
Clarithromycin 15mg/Kg/day in 2 divided doses 14 days
Omeprazole 1mg/Kg/day in 2 divided doses 1 month
Tinidazole 50mg/Kg/day (max 2g) 14 days
Clarithromycin 15mg/Kg/day in 2 divided doses 14 days
Omeprazole 1mg/Kg/day in 2 divided doses 1 month
Metronidazole 20mg/Kg/day in 2 divided doses 14 days
Clarithromycin 15mg/Kg/day in 2 divided doses 14 days

196
VOMITING
Vomiting and nausea are common sequelae of a multitude of
disorders.
 Can range from mild, self-limited illnesses to severe, life-
threatening conditions.
 Vomiting and nausea may or may not occur together, or may
be perceived at the same level of intensity.

Definitions
 Vomiting: a forceful oral expulsion of gastric contents
associated with contraction of the abdominal and chest wall
musculature.
 Nausea: The unpleasant sensation of the imminent need to
vomit,
o usually referred to the throat or epigastrium;
o a sensation that may or may not ultimately lead to the
act of vomiting.

 Regurgitation: The act by which food is brought back into the

mouth without the abdominal and diaphragmatic muscular
activity that characterizes vomiting.
 Retching: Spasmodic respiratory movements against a closed
glottis with contractions of the abdominal musculature
without expulsion of any gastric contents. This is referred to as
“dry heaves.”
 Rumination: Chewing and swallowing of regurgitated food
that has come back into the mouth through a voluntary
increase in abdominal pressure within minutes of eating or
during eating.

Differe ntial Diagnosis o f Vo miting

Vomiting is a symptom with a wide differential diagnosis, ranging
from lesions of the GI tract to systemic illnesses.

197
A detailed history, including dietary history, review of systems,
family history, medication history, medical history and surgical
history is important in the initial evaluation to identify a cause.
 Acute onset of vomiting with severe abdominal pain may
suggest a surgical origin; common associated symptoms
include localized or generalized abdominal tenderness, signs
of peritonitis, and absent or hyperactive bowel sounds.
 Vomiting is often characterized as non-bilious, bilious, or
bloody based on the content. Vomitus from the esophagus,
stomach, and first part of the duodenum usually consists of
ingested food and is clear or yellow.
 Bilious vomiting denotes the presence of bile and appears
light green to dark green. Bilious vomiting suggests obstruction
of the intestine beyond the ampulla of Vater until proven
otherwise.
 Hematemesis is the presence of blood in the vomitus. The
presence of bright red blood in emesis or gastric lavage
indicates active upper GI tract bleeding that may require
immediate attention.
o The presence of coffee-ground material in the vomitus
indicates that blood has been acted on by gastric acid.
o The presence of blood in vomitus in relationship to other
symptoms is also important.
o Blood that develops after initial episodes of retching or
emesis may be suggestive of a Mallory-Weiss tear

Vomiting is often described as projectile or non-projectile.

Projectile vomiting is commonly seen in gastric outlet obstruction,
such as pyloric stenosis, and in conditions that result in raised
intracranial pressure.
Management o f the acutely vomiting c hild
It is vital to assess the degree of dehydration and manage
accordingly.
Most children can be rehydrated with oral or nasogastric feeds
unless they have severe dehydration, in which case intravenous
resuscitation is essential.

198
 The child with vomiting should continue to be fed, (including
breastfeeding as appropriate) unless severely dehydrated.
 Specific treatment should be directed toward the underlying
etiology.
 The etiology should be sought, taking into account the child's
age, and whether the nausea and vomiting is acute, chronic,
or episodic.
 The consequences or complications of nausea and vomiting
(eg, fluid depletion, hypokalemia, and metabolic alkalosis)
should be identified and corrected.

 Antiemetics are typically are not recommended for vomiting

of unknown etiology, only useful for selected causes of
persistent vomiting, to avoid electrolyte abnormalities or
nutritional sequelae. They are not appropriate for treatment of
vomiting caused by anatomic abnormalities or surgical
abdomen; they are also contraindicated in infants.
 However, antiemetic drugs can be used cautiously in children
> 2 yr. Useful drugs include:
o Ondansetron: 0.15 mg/kg (maximum 8 mg) IV q 8 h or, if
the oral form is used, for children 2 - 4 yrs. 2 mg q 8 h; for
those 4 to 11 yr, 4 mg q 8 h; for those ≥ 12 yr, 8 mg q 8 h
 Ondansetron is a selective serotonin (5-HT3) receptor
blocker that inhibits the initiation of the vomiting reflex
in the periphery.
 A single dose of ondansetron is safe and effective in
children who have acute gastroenteritis and do not
respond to oral rehydration therapy (ORT).
 By facilitating ORT, this drug may prevent the need for
IV fluids or, in children given IV fluids, may help prevent
hospitalization.
 Typically, only a single dose is used because repeated
doses can cause persistent diarrhea.

Refere nces
Deborah M. Consolini, MD, Sidney Kimmel Medical College
of Thomas Jefferson University -Nausea and Vomiting in Infants and
Children, MSD MANUALS, 2019
199
 T. Matthew Shields, Jenifer R. Lightdale, MPH - Vomiting in
Children in: Pediatrics in Review, July 2018, 39 (7) 342-358;
Carlo Di Lorenzo, MD, Approach to the infant or child with
nausea and vomiting, 2019 UpToDate, B UK Li, MD (ED)

HYPERTROPHIC PYLORIC STENOSIS

Hypertrophic Pyloric Stenosis (HPS) is a condition that is caused
as a result of pylorus wall hypertrophy and hyperplasia, and
incomplete mechanical obstruction.
It is a common cause of gastric outlet obstruction in infants.

The cardinal features of HPS are non-bilious projectile vomiting

and visible peristalsis in the left upper abdominal quadrant.
 Bilious vomiting in this condition is rarely reported and may
create confusions in diagnosis
 Hypertrophic Pyloric Stenosis occurs in 2 to 3 infants per 1000
live births.
o It occurs much more commonly in males, with a male-
female ratio of 4:1 to 6:1.
o Up to one-third of cases of pyloric stenosis are seen in
first-born infants.
 Genetic factors play a role in pyloric stenosis;
o maternal history of pyloric stenosis is a risk factor
o Use of erythromycin in infants, especially within the first
weeks of life
o Maternal use of macrolide antibiotics during late
pregnancy and during breastfeeding
 The typical presentation is a 3- to 6-week-old infant with
progressive or intermittent vomiting after feeding.
o frequent vomiting, usually 10 to 30 min after feeding,
o The infant is hungry after vomiting.
o Infants with continued vomiting develop
hypochloraemic, hypokalaemic contraction alkalosis
and aciduria.

On phys ical examination

200
 Infant may be dehydrated
 Visible gastric peristalsis, from left to right across the
hypogastrium may be seen after a feed, just before the baby
vomits
 A pyloric mass, an olive-like mass may be felt below the liver
edge just lateral to the edge of the right rectus abdomens
muscle. It is best felt when the stomach is empty, just after a
vomit.

Investigations
 Bloods - FBC, RBS, venous blood gas (VBG),
o VBG shows a metabolic alkalosis (hypochloraemic,
hypokalaemic alkalosis) in those with metabolic
derangement
o Urea, creatinine and electrolytes
 Abdominal ultrasound will usually confirm the diagnosis.
o pyloric thickness of 4 mm (from 3 to 5 mm).
o pyloric length of 14 to 20mm, and
o pyloric diameter of 10 to 14 mm.
 Where U/S is not readily available plain abdominal films may
show the 'single bubble' of gastric dilatation (with little or no
air beyond the pylorus).
 Upper GI tract radiocontrast series can also be used to make
the diagnosis and have the added advantage of excluding
other obstructive lesions, such as antral web or malrotation
with volvulus.
o delayed gastric emptying
o peristaltic waves (caterpillar sign)
o elongated pylorus with a narrow lumen (string sign)
which may appear duplicated due to puckering of the
mucosa (double-track sign)
o the pylorus indents the contrast-filled antrum (shoulder
sign) and (tit sign) or base of the duodenal bulb
(mushroom sign)
o the entrance to the pylorus may be beak-shaped (beak
sign)

201
Differe ntial diagnosis
 Feeding problem or milk intolerance.
 Gastro-oesophageal reflux.
 Gastroenteritis.
 Duodenal atresia, oesophageal atresia or other bowel
obstruction in the newborn.
 Intestinal malrotation/acute midgut volvulus.

Init ial manageme nt

Vomiting of gastric contents leads to depletion of sodium,
potassium, and hydrochloric acid, which results in hypokalemic,
hypochloremic metabolic alkalosis.
 Infants with HPS require hospitalization and intravenous fluid
replacement therapy
 Nil by mouth
 A nasogastric tube is inserted all gastric losses via the NGT are
replaced with Hartmann’s solution or 0.9% saline via a second
infusion
 Insert intravenous cannula (and take bloods)
 Assess degree of dehydration
 Commence intravenous fluids
o Fluid rate = maintenance plus calculated deficit over 24
hours
o usually consisting of 5% dextrose in 0.45% saline (DNS) at
1.5 times maintenance rate.
 Surgical referral.

Refere nces
The Royal Children’s Hospital Clinical Practice Guidelines -
Pyloric stenosis, July 2019
Kalyan Ray Parashette, MD, Joseph Croffie, MD, MPH;
Vomiting -Pediatrics in Review Vol.34 No.7 July 2013

202
HAEMATO-ONCOLOGY

ANAEMIA

Definition
Haemoglobin (Hb) concentration or haematocrit two or more
standard deviations below the mean value for age and sex. See
table below:

Table xx Age-Specific Normative Red Blood Cell Values

Haemoglobin Haematocrit (%) Mean corpuscular
(g per dL) volume (fL)
Age Mean 2 SDs Mean 2 SDs Mean 2 SDs
below below below
mean mean mean
26 to 30 13.4 11.0 41.5 34.9 118.2 106.7
weeks’
gestation
28 weeks’ 14.5 NA 45 NA 120 NA
gestation
32 weeks’ 15.0 NA 47 NA 118 NA
gestation
Full term 16.5 13.5 51 42 108 98
(cord sample)
1 to 3 days 18.5 14.5 56 45 108 95
2 weeks 16.6 13.4 53 41 105 88
1 month 13.9 10.7 44 33 101 91
2 months 11.2 9.4 35 28 95 84
6 months 12.6 11.1 36 31 76 68
6 months to 2 12.0 10.5 36 33 78 70
years
2 to 6 years 12.5 11.5 37 34 81 75
6 to 12 years 13.5 11.5 40 35 86 77
12 to 18 yrs 14.5 13.0 43 36 88 78
(male)
12 to 18 yrs 14.0 12.0 41 37 90 78
(female)
Adult (male) 15.5 13.5 47 41 90 80
Adult (female) 14.0 12.0 41 36 90 80
NA = not available; SD = standard deviation.

203
 Adapted from Robertson J, Shilkofski N, eds. The Harriet Lane Handbook. 17th ed.
Philadelphia, Pa.: Mosby; 2005:337.

Causes
Decreased red cell production
 Deficiency of haematopoietic precursors ( Iron, Folate, vitamin
B12)
 Bone marrow failure (Malignant infiltration, Aplastic,
Congenital defects)

Increased red cell destruction

 Haemolytic disease
o Extracorpuscular defects ( immune mediated )
o Intracorpuscular defects ( Enzyme defect, membrane
defect, Globin defects)

Blood loss
 Acute
 Chronic

Clinical features
Symptoms associated with anaemia include

 Fever  Poor concentration

 Poor weight gain or  Palpitations
weight loss  Easy fatigability
 jaundice  Bruising episodes
 Pica  Bleeding episodes
 Headache

Signs associated with anaemia include:

 Pallor
 Fever
 Wasting
 Tachycardia
 Oedema
204
 Hepatosplenomegaly
 Lymphadenopathy
 Petechiae
 Purpura

Investigations
 Full blood count
 Peripheral blood smear
 Reticulocyte count
 Total and direct serum bilirubin levels
NOTE: These above first four tests are key in the characterisation
of the type of anaemia and possible cause

Other tests
 ESR – (erythrocyte sedimentation rate)
 Coombs – direct and indirect - tests
 Sickling test
 Bone marrow aspiration and bone marrow biopsy
 Coagulation studies ( APTT, PT/ INR, D-dimers, FDPs)
 Stool for occult blood and parasites and ova
 Urinalysis – dipstick, microscopy

Principles o f manage ment

 Treat the underlying pathology

 If the anaemia reoccurs, without establishing the underlying
cause, refer to the next level of care.
 Replace the appropriate blood product deficiency
 Include component of Rx for mild anaemia with haematinics.
This raise the Hb by 1g/dl in a month.
 Do not routinely give iron in sickle cell disease unless there is
documented iron deficiency.

Blood replacement formulae when a transfusion is indicated:

205
 Packed red blood cells in mL = body weight x deficit Hb x 4
 Whole blood in mL = Body weight x deficit Hb x 6
 Do not exceed a maximum 20ml/kg/day.
 Indicate amount of blood to be transfused on the drug chart

Refere nces
Nelson Textbook of Pediatrics, 18th edition, 2007
Blueprints Pediatrics, 4th edition, 2007
WHO/ UNICEF. Anaemia statement. 2004

Essential Revision Notes in Paediatrics, 2 nd edition, 2006

206
SICKLE CELL DISESASE – CVA
Stroke is one of the major complications of sickle cell disease
(SCD). Brain dysfunction occurs when oxygen supply to the brain
falls below a critical level based on need.

The approach to management depends on the specific brain

manifestations and the age of the patient. Symptoms of brain
ischemia include:
 Hemiparesis
 Facial weakness
 Severe headache
 Visual and language disturbances
 Seizures (especially focal seizures)
 Altered sensation

Differe ntials
Differentials include CNS infections (Meningitis/Encephalitis),
cerebral malaria, trauma with consequent subdural hematoma,
or intoxication, particularly if focal signs are not prominent.

Investigations
 Full blood count with differential & reticulocyte count
 Group and cross match
 Quantitative D- dimer if available
 Emergent CT or MRI/MRA (where available). Note that
contrast may exacerbate sickling. Discuss using contrast with
paediatric haematologist.
 Malaria parasite slide
 Blood culture
 Kidney and liver functions tests
 EEG (where indicated)

Management
 Admit patient to Intensive care Unit
 Adequate hydration at least two third of requirements to
guard against cerebral oedema.

207
 Immediate transfusion with packed cells within four hours and
repeat on day 4 or exchange transfusion.
 Hourly Glasgow Coma Scale monitoring
 Empirical antibiotics
 Anti-Epileptic drugs if seizures are present (Diazepam is usually
used at 0.2mg/kg).
 Start hyper transfusion protocol (See Appendix I).
 Commence hydroxyurea. The initial dose is 15/kg/day given
as a single daily dose. Dose escalation by 5mg/kg every 4- 6
weeks.
 Commence iron chelation therapy, deferasirox 10- 30mg//kg
once daily PO for 6 months.

Exc hange transfusion

Definition
 Full exchange transfusion (30ml/kg)
 Partial exchange (15ml/kg)

Indications
 Severe sickle cell chest syndrome or Girdle syndrome
 A new CVA
 Multi- organ failure, e.g. Associated with systemic fat
embolism.
 Fulminant priapism (> 4 hours) unresponsive to
pharmacological therapy.

Aim
 To reduce the HbS% level to < 30% over 2- 3 days if acutely ill,
when more rapid exchange may be appropriate.
 To keep Hb < 10 g/dL initially and by the end of the procedure
(or at steady state level in those with higher baseline Hb, e.g
HbSC patients of 11- 12g/dL
 To maintain a steady blood volume and Hb throughout the
exchange.

208
 There are two types of exchange transfusions. These are
manual and automated methods.
o A simple early method consists of removal of 500 mL
blood followed by infusion of 2 units of donor cells which
aims to achieve a 30% exchange in 90 minutes. More
recent techniques generally use 2 intravenous lines with
simultaneous or sequential withdrawal of SS blood and is
replaced by donor AA cells. However, although manual
procedures are effective in conducting exchange, they
are tedious and time-consuming.
o Machines performing centrifugal separation of red
blood cells provide a rapid and efficient method of
performing partial exchange transfusion. Two types of
machines are in common use, the discontinuous- and
continuous-flow models.
o Discontinuous-flow separators are small, mobile, simple
to operate, relatively cheap, and require only 1
intravenous line. Discontinuous-flow cell separators act
as batch processors, pumping blood from the patient’s
arm, centrifuging it to separate red cells from the
plasma, discarding the red cells, and returning the
plasma to the patient along with donor cells.
o Continuous-flow separators are more complex and
expensive, and more efficient but require 2 intravenous
lines with large needles or catheters capable of carrying
the large flows. Continuous-flow separators use 2
intravenous lines, one extracting blood and the other
returning the processed blood to the patient. Controlling
rates of inflow and outflow ensures that the patient’s
blood volume does not vary during the procedure.
Supplies for both cell separator techniques are
considerably more expensive than manual methods but
require less professional time.

Preliminary investigations
 Full Blood Count
 Hb electrophoresis (not urgent at first exchange)
 Group and Cross match

209
 Urea, electrolytes, Creatinine, liver function tests, Serum
calcium.
 Arterial blood gases in those with symptoms suggestive of
Acute Chest Syndrome or girdle syndrome.
 Hepatitis B and C and HIV.

Hydroxyurea
Mechanism: Hydroxyurea is an antineoplastic that may cause
inhibition of DNA synthesis by acting as a ribonucleotide
reductase inhibitor. It increases fetal haemoglobin, reduces
neutrophils, and alters adhesion of red blood cells to
endothelium, increasing water content of red blood cells and
increases deformability of sickled cells.
Indications: All children above nine months of age with SCD
should be on hydroxyurea.
Exclusions: acute liver disease, history of severe hydroxyurea
toxicity or hypersensitivity, pregnancy or sexually active and
unwillingness to use contraception.

Baseline investigations :
 FBC with Differential count.
 Hb electrophoresis with quantitative Hb F %.
 Chemistry profile.
 Liver function tests (AST, ALT).
 Renal function tests (BUN, creatinine)
Consult with paediatric haematologist for appropriate work- up
before initiating a patient on hydroxyurea.

Dosing:
 Initial dose 15/kg/day given as a single daily dose.
 Dose escalation by 5mg/kg every 4- 6 weeks.
 Increase dose until ANC 2, 000- 3, 000 achieved or
35mg/kg/day dose achieved or evidence of haematological
toxicity.

210
 Liquid formulation can be prepared by compounding
pharmacy using published guideline.

Transcranial Do pple r Ultraso und

A transcranial Doppler (TCD) ultrasound examination is a
nonivasive technique that assesses blood flow within the circle of
Willis and the vertebrobasilar system.
It can identify children with SCD who are at high risk for stroke by
documenting abnormal high blood flow velocity.
In children with SCD, screen annually with TCD according to
methods employed in the STOP studies, beginning at age 2 years
and continuing until at least age 16. In children with conditional
(170- 199cm/sec) or elevated (>200cm/sec) TCD results need to
be commenced on chronic transfusion therapy aimed at
preventing stroke.’

Refere nces
Evidence- based Management of Sickle Cell Disease, Expert
Panel Report, 2014. http://nhlbi.gov/guidelines
Adams R.J. et al Prevention of a first stroke by transfusions in
children with sickle cell anaemia and abnormal results on
transcranial Doppler
Anderson R, Cassell M, Mullinax GL, Chaplin H Jr. Effect of
normal cells on viscosity of sickle-cell blood: in vitro studies
and reports of six years’ experience with a prophylactic
program of “partial exchange transfusion”. Arch Intern Med.
1963; 111:286–294.
Janes SL, Pocock M, Bishop E, Bevan DH. Automated red cell
exchange in sickle cell disease. Br J Haematol. 1997;
97(2):256–258.

211
HAEMOPHILIA
Haemophilia is a congenital bleeding disorder affecting 1 in
every 10,000 males caused by deficiency of factor VIII
(haemophilia A) or factor IX (haemophilia B) or rarely factor XI
(haemophilia C). Haemophilia A and B are X-linked, whereas
haemophilia C is autosomal recessive. Haemophilia A is the most
common.

Diagnostic Testing
 Prolonged APTT with normal PT and normal platelet count is
suggestive.
 Confirm with Factor VIII or Factor IX assay.

Severity Factor Level

Severe < 1%
Moderate 4%
Mild 5- 30%

NOTE: Normal factor level ranges from 50- 200%.

Prophylaxis
All patients should receive prophylaxis using the low dose
protocol at 15-20 iu/kg once weekly subcutaneously.

Inhibitor Sc reening
Where available, screening for inhibitors should be performed.
Mixing study is a qualitative inhibitor screen. Bethesda assay is
the gold standard.

Treatme nt of bleeding
Patients with haemophilia can have spontaneous bleeding and/
or excessive bleeding with trauma. It’s imperative to treat
haemophilia patients with factor within 30 minutes of
presentation first and then consider diagnostic testing.
 Factor VIII deficiency (Haemophilia A)
o 1 unit/kg increases Factor VIII activity by 2%
o Give 30 units/kg of Factor VIII for an acute mild bleed.
o Give 50 units/kg of Factor VIII for an acute major bleed.
212
 o Half-life of Factor VIII is 8- 12 hours. Major bleeds require
8- 12 hour dosing.
o Discuss treatment plan with paediatric Haematologist.
 Factor IX deficiency (Haemophilia B)
o 1 unit/kg increases Factor IX activity by 1%.
o Give 50 units/kg of Factor IX activity for an acute minor
bleed.
o Give 100 units/kg of Factor IX for an acute major bleed.
o Half- life of Factor IX is 18- 24hours. Major bleeds require
at 24 hour dosing.
NOTE: It’s important to round up both Factor VIII and
Factor IX to a unit vial dose whenever possible to avoid
wastage. E.g. if unit vial is 500 units and patient’s dose is
400 units, order 500 units Factor VIII.

 Where Factor VIII or Factor IX is unavailable, or not

immediately available in emergency situations give plasma
(Fresh Frozen Plasma- FFP) at 10- 20mL/kg IV to run over 1 hour.

Suppo rtive care

 Ice for 20 minutes to both haemoarthosis and soft tissue
bleeds.
 Immobiliastion for mucosal bleeding and dental extraction
may also add tranexamic acid 10mg/kg/dose every 8 hours
for 2- 8 days.
 Head trauma should have an emergent CT scan after the first
dose of factor given even if there are no neurologic signs.

Immunisations
Haemophilia patients, young and old, can and should receive
recommended immunisations for their age group. Subcutaneous
vaccines are generally safer and more acceptable. Were
necessary, intramuscular vaccines should be given using a small
needle such as 24 gauge needles (or smaller) in a larger muscle.
 It might be necessary to administer factor before immunizing
a known Haemophilia patient. The best method is to schedule
vaccination at or around the same time as the factor dose for
patients on prophylactic factor therapy.
213
 After immunization, apply gentle pressure and treat with ice to
reduce bleeding.
 Always warn about the risk of hematoma.
 Provide pain relief; Acetaminophen is the drug of choice.

Emicizumab
Emicizumab is a novel bispecific monoclonal antibody which
mimics coagulation factor VIII and therefore, is capable of
promoting the activation of FX by FIXa, resulting in downstream
haemostasis at the site of bleeding in patients with haemophilia
A who have decreased or no circulating levels of FVIII.
Indications

Emicizumab is indicated for routine prophylaxis of bleeding

episodes in patients with haemophilia A with factor VIII inhibitors.

Emicizumab is effective in prophylactic treatment among adult

and paediatric patients, with or without inhibitors. The priority for
emicizumab and the WFH in Zambia is as follows:
A. Children under 12 years of age, diagnosed with inhibitors.
B. Patients with haemophilia 12 years and above diagnosed
with inhibitors.
C. Children under 12 years of age, without inhibitors based on
bleeding patterns.

Dosage

The recommended dose is 3 mg/kg once weekly for the first 4

weeks (loading dose), followed by 6 mg/kg once monthly
(maintenance dose), administered as a subcutaneous injection.
Administration of Emicizumab will only be done at the University
Teaching Hospital- Children’s Hospital Haemophilia Treatment
Centre (HTC) in Lusaka at the moment.
Storage

Opened vials:
 Single use
 Discard any unused solution remaining after administering
dose.

214
Unopened vials:

 Storage in refrigerator at 2- 8ᵒC in original carton to protect

from direct light
 Do not freeze
 Do not shake
 If necessary, unopened vials may be stored at room
temperature and then returned to refrigeration; temperature
should not exceed 30ᵒ C for up to 7 days.

Refere nces
1. Guidelines for the management of haemophilia, World
Federation of Hemophilia (WFH) second edition 2012.
www.wfh.org

2. Johnny N Mahlangu and Anne Gilham, 2008. Guidelines for

the treatment of Haemophilia in South Africa, SAMJ, Vol 99,
No. 2.

215
IMMUNE THROMBOCYTOPENIA (ITP)

Introduction
Immune thrombocytopenia (ITP) is a syndrome in which platelets
become coated with autoantibodies to platelet membrane
antigens, resulting in splenic sequestration and phagocytosis by
mononuclear macrophages. The resulting shortened life span of
platelets in the circulation, together with incomplete
compensation by increased platelet production by bone
marrow megakaryocytes, results in a decreased number of
circulating platelets (thrombocytopenia).

Etiology
In children, most cases of immune thrombocytopenia (ITP) are
acute, manifesting a few weeks after a viral illness. In adults,
most cases of ITP are chronic, manifesting with an insidious onset,
and occur in middle-aged women. These clinical presentations
suggest that the triggering events may be different. However, in
both children and adults, the cause of thrombocytopenia
(destruction of antibody-coated platelets by mononuclear
macrophages) appears to be similar and involve.
 Autoantibody stimulation
 Autoantibody specificity
 Role of the spleen
 Platelet destruction

Signs and Sympto ms

ITP manifests as a bleeding tendency, easy bruising (purpura), or
extravasation of blood from capillaries into skin and mucous
membranes (petechiae). Although most cases of acute ITP,
particularly in children, are mild and self-limited, intracranial
hemorrhage may occur when the platelet count drops below 10
× 109/L (< 10 × 103/µL); this occurs in 0.5-1% of children, and half
of these cases are fatal.

ITP is a primary illness occurring in an otherwise healthy person.

Signs of chronic disease, infection, wasting, or poor nutrition

216
indicate that the patient has another illness. Splenomegaly
excludes the diagnosis of ITP.
An initial impression of the severity of ITP is formed by examining
the skin and mucous membranes, as follows:
 Widespread petechiae and ecchymoses, oozing from a
venipuncture site, gingival bleeding, and hemorrhagic bullae
indicate that the patient is at risk for a serious bleeding
complication.
 If the patient's blood pressure was taken recently, petechiae
may be observed under and distal to the area where the cuff
was placed and inflated.
 Suction-type electrocardiograph (ECG) leads may induce
petechiae.
 Petechiae over the ankles in ambulatory patients or on the
back in bedridden ones suggest mild thrombocytopenia and
a relatively low risk for a serious bleeding complication.
Findings suggestive of intracranial hemorrhage include the
following:

 Headache, blurred vision, somnolence, or loss of

consciousness.
 Hypertension and bradycardia, which may be signs of
increased intracranial pressure.
 On neurologic examination, any asymmetrical finding of
recent onset.
 On fundoscopic examination, blurring of the optic disc
margins or retinal hemorrhage.

Diagnosis
On complete blood cell count, isolated thrombocytopenia is the
hallmark of ITP. Anemia and/or neutropenia may indicate other
diseases. Findings on peripheral blood smear are as follows:

 The morphology of red blood cells (RBCs) and leukocytes is

normal.
 The morphology of platelets is typically normal, with varying
numbers of large platelets.

217
 If most of the platelets are large, approximating the diameter
of red blood cells, or if they lack granules or have an
abnormal color, consider an inherited platelet disorder.
Many children with acute ITP have an increased number of
normal or atypical lymphocytes on the peripheral smear,
reflecting a recent viral illness. Clumps of platelets on a
peripheral smear prepared from ethylenediaminetetraacetic
acid (EDTA)–anticoagulated blood are evidence of
pseudothrombocytopenia. This diagnosis is established if the
platelet count is normal when repeated on a sample from
heparin-anticoagulated or citrate-anticoagulated blood.
No single laboratory result or clinical finding establishes a
diagnosis of ITP; it is a diagnosis of exclusion. The differential
diagnosis includes such other causes of thrombocytopenia as
leukemia, myelophthisic marrow infiltration,
myelodysplasia, aplastic anemia, and adverse drug reactions.
Pseudothrombocytopenia due to platelet clumping is also a
diagnostic consideration.

Aspects of bone marrow aspiration and biopsy are as follows:

 The value of bone marrow evaluation for a diagnosis of ITP is
unresolved.
 Biopsy in patients with ITP shows a normal-to-increased
number of megakaryocytes in the absence of other
significant abnormalities.
 In children, bone marrow examination is not required except
in patients with atypical hematologic findings, such as
immature cells on the peripheral smear or persistent
neutropenia.
 In adults older than 60 years, biopsy is used to exclude
myelodysplastic syndrome or leukemia.
 In adults whose treatment includes corticosteroids, a baseline
pretreatment biopsy may prove useful for future reference, as
corticosteroids can change marrow morphology.
 Biopsy is performed before splenectomy to evaluate for
possible hypoplasia or fibrosis.
 Unresponsiveness to standard treatment after 6 months is an
indication for bone marrow aspiration.
218
Management
ITP has no cure, and relapses may occur years after seemingly
successful medical or surgical management. Most children with
acute ITP do not require treatment, and the condition resolves
spontaneously.
Treatment is as follows:

 Corticosteroids remain the drugs of choice for the initial

management of acute ITP.
 Oral prednisone, IV methylprednisolone, or high-dose
dexamethasone may be used.
 IV immunoglobulin (IVIG) has been the drug of second choice
for many years .
 For Rh(D)-positive patients with intact spleens, IV Rho
immunoglobulin (RhIG) offers comparable efficacy, less
toxicity, greater ease of administration, and a lower cost than
IVIG.
 RhIG can induce immune hemolysis (immune hemolytic
anemia) in Rh(D)-positive persons and should not be used
when the hemoglobin concentration is less than 8 g/dL.
 Sporadic cases of massive intravascular
hemolysis, disseminated intravascular coagulation
(particularly in elderly individuals), and renal failure have been
reported with RhIG.
 Rituximab is third-line therapy.
 Platelet transfusions may be required to control clinically
significant bleeding but are not recommended for
prophylaxis.
 If 6 months of medical management fails to increase the
platelet count to a safe range (about 30,000/µL),
splenectomy becomes an option.
 Thrombopoietin receptor agonists (ie, eltrombopag,
romiplostim) may maintain platelet counts at safe levels in
adults with chronic ITP refractory to conventional medical
management or splenectomy.
Pregnant adolescents require special consideration for delivery,
as follows:

219
 If the platelet count is greater than 50 × 109/L (>50 × 103/µL),
the risk of serious hemorrhage is low, but beginning oral
prednisone a week before delivery is a reasonable
precaution.
 If the platelet count is less than 50 × 109/L (50 × 103/µL) before
delivery, treatment with oral prednisone and IVIG is
recommended.
 Avoiding the use of IV RhIG in this situation until safety data
are available is advisable.
 Rarely, splenectomy may be required to manage acute
hemorrhage.

220
APLASTIC ANEMIA

Introduction
Aplastic anemia is a syndrome of bone marrow failure
characterized by peripheral pancytopenia and marrow
hypoplasia (see the image below). Although the anemia is often
normocytic, mild macrocytosis can also be observed in
association with stress erythropoiesis and elevated fetal
hemoglobin levels.

Signs and symptoms

The clinical presentation of patients with aplastic anemia
includes symptoms related to the decrease in bone marrow
production of hematopoietic cells. The onset is insidious, and the
initial symptom is frequently related to anemia or bleeding,
although fever or infections may be noted at presentation.
Signs and symptoms of aplastic anemia may include the
following:

 Pallor
 Headache
 Palpitations, dyspnea
 Fatigue
 Foot swelling
 Gingival bleeding, petechial rashes
 Overt and/or recurrent infections
 Oropharyngeal ulcerations

Etiology
The theoretical basis for marrow failure includes primary defects
in or damage to the stem cell or the marrow
microenvironment. The distinction between acquired and
inherited disease may present a clinical challenge, but more
than 80% of cases are acquired. Clinical and laboratory
observations suggest that acquired aplastic anemia is an
autoimmune disease.

Conge nital or inherited causes

221
Congenital or inherited causes of aplastic anemia are
responsible for at least 25% of children with this condition and for
perhaps up to 10% of adults. Patients may have dysmorphic
features or physical stigmata, but marrow failure may be the
initial presenting feature.
Fanconi anemia

Fanconi anemia is characterized by the following:

 Multiple congenital anomalies (60-75%): Short stature,
abnormal skin pigmentation, malformations of the thumbs
with or without dysplastic or absent radii, as well as
microphthalmos and malformations of the heart, kidneys,
intestines, and ears.
 Bone marrow failure: Thrombocytopenia, leucopenia, or
aplastic anemia; most patients with Fanconi anemia have
bone marrow failure by adulthood.

Dyskeratosis congenita

Dyskeratosis congenita is characterized by the diagnostic

physical triad of dysplastic nails, lacy reticular pigmentation of
the upper torso, and oral leukoplakia. However, over the past
decade, it has been increasingly recognized that patients may
have dyskeratosis congenita without the triad.

Familial aplastic anemia

This is an isolated aplastic anemia.

Cartilage-hair hypoplasia

Cartilage-hair hypoplasia, characterized by the following:

 Short stature with short and bowed limbs

 Sparse, lightly pigmented hair
 Variably severe immune deficiency
 Anemia during childhood
 Hematopoietic malignancies, as well as malignancies of the
skin, eyes, and liver
 Gastrointestinal malformations and malabsorption

Pearson syndrome
222
Pearson syndrome causes sideroblastic anemia and exocrine
pancreatic dysfunction.
Thrombocytopenia-absent radius syndrome

Thrombocytopenia-absent radius (TAR) syndrome is

characterized by bilateral absence of the radii with presence of
the thumbs, as well as thrombocytopenia. Other congenital
anomalies can also occur (e.g. cardiac disease, skeletal
anomalies, urogenital anomalies).

Shwachman-Diamond syndrome

Shwachman-Diamond syndrome is inherited in an autosomal

recessive manner. This disease is characterized by dysfunction of
the exocrine pancreas with malabsorption and growth failure, as
well as cytopenias of single or multiple lineage. Patients with
Shwachman-Diamond syndrome also have an increased risk of
MDS and AML.

Dubowitz syndrome

Dubowitz syndrome is characterized by intrauterine growth

retardation, extremely short stature, and wizened facial
appearance. Patients also have microcephaly and mild
developmental delay. Dubowitz syndrome is also associated
with eczema, immune deficiency, and aplastic anemia.
Malignancy is more common with this disorder, particularly
lymphoma and neuroblastoma.

Diamond-Blackfan anemia

Diamond-Blackfan anemia (DBA) is characterized by a

normochromic macrocytic anemia that can be isolated, or it
can be associated with growth retardation or congenital
malformation in the upper limbs, heart, and genitourinary
systems. In a small minority of patients, DBA can progress to
aplastic anemia. Approximately 50% of cases are inherited from
a parent and about 50% result from de novo mutations.

Acquired causes
Acquired causes of aplastic anemia (80%) include the following:
 Idiopathic factors
223
 Infectious causes, such as hepatitis viruses, Epstein-Barr virus
(EBV), human immunodeficiency virus (HIV), parvovirus, and
mycobacteria.
 Exposure to ionizing radiation.
 Exposure to toxic chemicals, such as benzene or pesticides.
 Transfusional graft versus host disease (GVHD)
 Orthotopic liver transplantation for fulminant hepatitis.
 Pregnancy
 Eosinophilic fasciitis
 Anorexia
 Severe nutritional deficiencies (B12, folate)
 Paroxysmal nocturnal hemoglobinuria (PNH)
 MDS
 Acute lymphoblastic leukemia (ALL) rarely.
 Drugs and elements (e.g., chloramphenicol, phenylbutazone,
gold) may cause aplasia of the marrow.

Diagnosis
Laboratory testing for suspected aplastic anemia includes the
following:
 Complete blood count
 Peripheral blood smears
 Hemoglobin electrophoresis and blood-group testing
 Biochemical profile
 Serology for hepatitis and other viral entities
 Autoimmune-disease evaluation for evidence of collagen-
vascular disease.
 Kidney function studies
 Liver function studies
 Transaminase, bilirubin, and lactate dehydrogenase levels.

Procedures

224
Bone marrow biopsy is performed in addition to aspiration to
assess cellularity qualitatively and quantitatively. Bone marrow
culture may be useful in diagnosing mycobacterial and viral
infections; however, the yield is generally low.

Management
Severe or very severe aplastic anemia is a hematologic
emergency, and care should be instituted promptly. Clinicians
must stress the need for patient compliance with therapy. The
specific medications administered depend on the choice of
therapy and whether it is supportive care only,
immunosuppressive therapy, or hematopoietic cell
transplantation.
Pharmacotherapy

The following medications are used in patients with aplastic

anemia:
 Immunosuppressive agents (e.g. cyclosporine,
methylprednisolone, equine antithymocyte globulin, rabbit
antithymocyte globulin, cyclophosphamide, alemtuzumab).
 Hematopoietic growth factors (eg, eltrombopag ,
sargramostim, filgrastim).
 Antimetabolite (purine) antineoplastic agents (eg,
fludarabine).
 Chelating agents (eg, deferoxamine, deferasirox).

Nonpharmacotherapy

Nonpharmacologic management of aplastic anemia includes

the following:
 Supportive care
 Blood transfusions with blood products that have undergone
leukocyte reduction and irradiation.
 Hematopoietic cell transplantation

225
226
APPROACH TO DIAGNOSIS AND INVESTIGATION OF
COMMON MALIGNANCIES
Causes of malignancy
Risk factors for cancer can be broadly classified as:

 Genetic causes
 Environmental causes

Clinical features
In the first two years of life, embryonic tumours tend to be
common. Examples include Nephroblastoma, Retinoblastoma,
Neuroblastoma, Teratoma, Rhabdomyosarcoma,
Medulloblastoma.

From 2-5 years of age, the embryonic tumours combine with

Acute Lymphoblastic Leukaemia, Non-Hodgkin’s Lymphoma,
and Glioma.
In adolescents Hodgkin’s disease, Bone, Gonadal and
Connective tissue tumours predominate

Commo n signs and sympto ms of cancer in c hildre n

 Abdominal mass
 Persistent generalized lymphadenopathy
 More than one abnormal hematopoietic lineage
 Specific neurologic deficit
 Increased intracranial pressure
 Proptosis
 White pupillary reflex
 Vaginal bleeding or mass
 Joint or bone swelling or pain

Uncommo n symptoms and signs of cancer in

childre n
 Superior vena cava syndrome
 Subcutaneous nodules
 Chronic diarrhoea

227
 Failure to thrive
 Skin lesions

History
General: a single symptom might cause patient to seek medical

Attention (e.g. Pain, mass, Bleeding, recurrent fevers, weakness)

Specific: s ymptoms to look for in the histo ry

CNS R/S MSS
Headache Cough Swelling
Fits Difficulties breathing Wasting
Blindness Haemoptysis Bone/joint pain
Vomiting Neck Non healing ulcer
Stiff neck Swelling Skin lesion
Confusion Engorgement Bleeding spots

GIT GUT OTHERS

Abdominal mass Pelvic/suprapubic mass Malaise
Diarrhoea Dysuria Anorexia
Easy satiety Haematuria Nights sweats
Dysphagia Urinary retention Fever
Poor appetite
Melaena

PHYSICAL EXAMINATION
CNS R/S MSS
Mentation Tachypnoea Skin lesion
Cranial palsy Reduced breath sounds Wasting
Paraplegia/Paraparesis Stridor Oedema
Meningismus Haemoptysis
Vomiting
Confusion
GIT GUT NECK

228
 Abdominal mass Proteinuria Lymphadenopathy
Hepatomegaly/spleno Haematuria Engorged vessels
megaly
Visible distension of
vessels

Commo n cancers on t he Haematology-Oncology

Unit
 Nephroblastoma
 Retinoblastoma
 Leukaemia
 Hodgkin’s lymphoma

Investigations
Blood Tests
 Complete Blood count
 Peripheral Smear
 Erythrocyte sedimentation rate
 Liver and Kidney Test
 Retroviral test

Imaging St udies
 Chest radiography: Postero-anterior and lateral views to
o Assess mediastinal masses
o Evaluate the airway
o Exclude pulmonary parenchymal lesions

 Ultrasonography: Abdomen, Pelvis, Eye, Cranial to identify site

and disease extent

 Computed tomography
o CT scans of the chest, abdomen, and pelvis can be
used to stage lesions

229
 o Chest CT scan is indicated to assess for the degree of
tracheal compression
o Head CT scans assist in excluding mass lesions and
possible meningeal involvement among individuals with
CNS disease.

 Bone scanning and skeletal surveys

o When additional symptoms are present, these tests help
in identifying additional sites of disease.

 Echocardiography
 can be obtained as baseline findings before patients are
given chemotherapy with anthracyclines, which can cause
cardiomyopathy.

Procedures
Bone marrow aspiration/biopsy
 Biopsy is necessary to assess for evidence of bone marrow
involvement in patients with lymphomas. Sites for BMA include
the anterior iliac crest, posterior iliac crest, sternum and tibia
(in infants).

Biopsy
 For patients with a mass, tissue is generally available from
resection or intra-operative biopsy.
 As an alternative, a diagnosis may be made by using pleural
fluid or ascetic fluid.

Lumbar puncture
 To determine the CSF cell count and differential: This test is
done to assess CNS involvement, the presence of which alters
therapy.

Management
Once diagnosis is made either histologically of clinically, stage
the patient according to the staging used for that particular
tumour. Consult the protocol to determine treatment modality
which could be either one or a combination of two or all three:
230
 Surgery
 Chemotherapy
 Radiation
Treatment should aim at either:
 Cure, or
 Palliation

MANAGEMENT OF PAINFUL CRISIS

Pain is a common, underreported and under- diagnosed
problem for hospitalised children wide, especially for infants and
mentally challenged children.

Assess the intensity of the pain by using: “face pain” intensity

scale or visual analogy scale (See appendix), multi- dimensional
scale, clinical acumen.
WHO analgesic ladder advocates for a step- wise approach to
treating pain. At every step of the analgesic ladder non- opioid
analgesics form the basis of the pain management.
Paracetamol and NSAIDs (if not contraindicated) should always
therefore be prescribed with opioid analgesia.
Treat pain aggressively and promptly. Begin analgesic
management within 15 minutes of triage or within 30 minutes of
registration.
Severity Management
Reassurance, warm packs, reposition, massage,
distraction (stories, play)
Mild Child: Paracetamol 15mg/kg QID
Adult: Paracetamol 1g QID
As for mild pain, PLUS
Moderate Child: Ibuprofen 5mg/kg TDS OR Diclofenac 1mg/kg TDS
Adult: Ibuprofen 400mg TDS OR Diclofenac 100mg TDS
As for moderate pain PLUS
Severe Child: Oral morphine 0.2- 0.3mg/kg 4 hourly as needed
Adult: Oral morphine 5- 10mg 4 hourly as needed

231
Step I:

Establish Intravenous (IV) access.

Begin with hydration to facilitate circulation of blood: use 5%
Dextrose normal saline IVF (1.5 x maintenance or 2,250 ml/
square metre/day) if no cardiopulmonary compromise.

Step II:

Assess for cause of pain and complications. Use pain assessment

tool (See appendix). Ask the patient to rate the pain as
mild/moderate/severe. Ask about site, severity and duration of
pain (usually bones/spine/abdomen). Conduct a complete
systemic examination to look for complications.

Step III:

 Give analgesia as per guidelines (Table above).

 If febrile, commence IV antibiotics.
 Monitor pulse, respiratory rate and oxygen saturations.
 Look out for side effects of morphine- nausea/vomiting,
pruritis, drowsiness and constipation. Always prescribe
laxatives (stool softener + stimulant) when starting opioids.

Step IV

 Stop IV fluids when the patient is stable, and pain controlled

 Weigh the child daily
 Avoid fluid overload

232
INFECTIOUS DISEASE

MALARIA
Malaria is one of the top five diseases causing morbidity and
mortality in Zambian children. Malaria is a febrile illness caused
by infection with the plasmodium falciparum parasite which is
transmitted from person to person by mosquitoes. Malaria is
diagnosed by examining a patient’s stained blood slide through
a microscope (MPS). A rapid malaria diagnostic test (RDT) for
malaria antigen can be done in the examination room and
results are available within 15 minutes. It is also important to also
do a full blood count (FBC). Left untreated, Malaria can be fatal

Uncomplicated malaria symptoms and signs are as follows:

 Fever
 Headache
 Abdominal pain
 Nausea
 Diarrhoea and Vomiting
 General body pains
 Weakness

Simple malaria is treated with Artemether 20 mg and

Lumefantrine 120 mg ) given with food as follows:
 stat,
 after 8 hrs,
 then 12 hourly doses on day 2 and day 3.

If the drug is spat out or vomited within 30 minutes, the dose

should be repeated. If more than two consecutive episodes of
vomiting occur, parenteral Artesunate should be administered.
WEIGHT (Kg) AGE (approx. DOSE (number of tabs of AL,
in yrs.) Artemether 20, Lumefantrine 120 mg)
5-<15 2months - 1 tab
3yrs
15-25 3-8yrs 2 tab
25-35 9-12yrs 3 tab

233
 >35 >12yrs 4 tab

Severe Malaria
Severe forms of malaria, may involve the
 Brain (cerebral malaria),
 Kidney (black water fever, acute kidney injury),
 Lungs (pulmonary oedema) ,
 Blood (severe anaemia)
 Cardiovascular system (shock).

The sympto ms and signs o f se vere malaria include

 Convulsions
 Changes in behaviour
 Reduced level of consciousness
 Coma
 Severe pallor
 Respiratory distress
 Jaundice
 Shock
 Coca Cola coloured urine
 Reduced urine output
 Bleeding tendency
 Generalized weakness
If a blood slide or RDT is negative then only children with severe
disease or those with severe anaemia should get presumptive
treatment immediately then to send a sample to the National
Malaria Elimination Centre for further analysis.

Investigations for severe malaria

 Thick and thin malaria parasite smear or RDT
 FBC
 Dextrostix
 U&Es and Creatinine
 LFTs

234
 Blood gases
 CXR
 Urinalysis
 Do LP to exclude meningitis if indicated and if no signs of ICP

Severe malaria = fever + any of t he follo wing

 Impaired level of consciousness (AVPU= V, P, U or low GCS
<11,Blantyre Coma Scale <3).
 Unable to drink/feed.
 Respiratory distress with acidotic breathing.
 Severe anaemia Hb < 5 g/dl.
 Hypoglycaemia blood glucose < 3 mmol/L.
 2 or more convulsions within 24 hours or a single episode of
status epilepticus.
 Plasma bicarbonate of < 15mmol/l 0r PH<7.35.
 Serum creatinine >3 times from baseline for age.
 Bleeding tendency- recurrent or prolonged bleeding from the
nose, gums, melaena, haematemesis, venepuncture sites.
 Shock – impaired perfusion (Capillary refill time of >3 seconds,
cool peripheries).
If yes, those >20kg should be given Artesunate 2.4mg/kg of body
weight IV or IM given on admission (time=0), then at 12hrs and
24hrs, then re-assess the patient and repeat MPS.

For patients with weight <20kg give Artesunate at 3mg/kg IV or

IM
After initial parenteral treatment for a minimum of 24hrs, once
the patient regains consciousness and can take medications
orally, and repeat MPS is negative, discontinue parenteral
therapy and commence full course of Artemether Lumefantrine
After initial parenteral treatment for a minimum of 24 hrs, and the
patient has not regained consciousness or cannot take
medications orally, and repeat MPS is positive continue
parenteral therapy once a day for a maximum of 6 days and
commence full course of Artemether Lumefantrine .

235
Repeat the blood slide every 24 hours until there is zero
parasitaemia.
If MPS remains positive despite full course of treatment, to consult
the National Malaria Elimination centres and to send DBS on filter
paper for further analysis.

There should be an interval of at least 8hrs between the last dose

of artesunate and the first dose of artemether lumefantrine.
In the absence of IV Artesunate, artemether may be used at
3.2mg/kg IM loading dose, then maintain with 1.6mg/kg OD for
5days

OR Alternative 2nd line drug for severe malaria is QUININE IV

 Loading dose of 20mg/kg body weight diluted in 10ml/kg of
5% or 10% dextrose by IV infusion over 4 hours. After eight
hours hours, give a maintenance dose at 10mg/kg body
weight over 4 hours, and repeat every eight hours until patient
can swallow.
 Then use oral quinine at 10mg/kg body weight every eight
hours to complete a seven-day course of treatment
 Treat hypoglycaemia with 5 ml/kg 10% dextrose IV, followed
by a continuous infusion of either 5% or 10% dextrose
 Maintenance fluids/feeds and supportive oxygen by mask
 If weak pulse and CRT > 3 sec, give 20 mls/kg normal saline,
up to 40mls/kg/day. Use blood for resuscitation, if Hb < 5g/dl
at 20 ml/kg whole blood).
 Use inotropes when there`s poor response to fluid boluses
 Treat convulsions as indicated in the algorithm for treatment
of convulsions.

Refere nces
1. Guidelines for the diagnosis and treatment of Malaria in
Zambia, Fourth edition, 2014

2. Artesunate versus quinine for treating severe Malaria

(review). The Cochrane Collaboration. Published by John
Wiley &Sons, Ltd.

236
3. Guidelines for the diagnosis and treatment of Malaria in
Zambia, Fifth edition, 2017

TYPHOID (ENTERIC FEVER) - DR MULENGA

Causes

Salmonella typhi, Salmonella paratyphi

Clinical features
High grade fever, coated tongue, anorexia, vomiting,
hepatomegaly, diarrhoea, toxicity, abdominal pain, pallor,
splenomegaly, constipation, headache, jaundice, obtundation,
ileus, intestinal perforation.

Diagnosis
 Blood culture
o Ideally 1st week of symptoms - Blood culture
o 2nd week of symptoms - Urine culture
o 3rd week of symptoms - Stool culture
NOTE: As most patients present late, all three cultures
should be taken on admission.
 Widal test
o A single Widal test may be positive in only 50% cases in
endemic areas
o Serial tests may be required

 Mainstay of diagnosis remains clinical

o Any high fever of >72 hours duration (with
aforementioned features), especially with no localizing
upper respiratory signs or signs of meningitis or malaria
must be suspected of typhoid and managed
accordingly.

 Full Blood count (White cell count)

237
 o Leucopoenia (WCC < 4 x109/litre) with a left shift in
neutrophils may be seen in a third of children; young
infants may also commonly present with leukocytosis.

 Other serological tests (expensive)

o Dot-Elisa
o Coagglutination
o TubexR

Management
 Early diagnosis and instituting appropriate supportive
measures and specific antibiotic therapy is the key to
appropriate management
 Adequate rest, hydration, correction of fluid-electrolytes and
nutrition
 Anti-pyretic therapy (paracetamol) as required if fever > 39 oC
 Antibiotic therapy
o 1st line therapy- ciprofloxacin (while awaiting culture
results). Alternative  Azithromycin
o 2nd line (drug resistant) – imipenem

 Monitor vital signs to recognise surgical emergencies

Intestinal haemorrhage (<1%) and perforation (0.5-1%)
Pain- sudden increase in abdominal pain
o Pulse Rate -a sudden rise in pulse rate,
o BP- hypotension,
o Temperature
o Respiratory rate
o Plain –X-ray abdomen should be done

Intestinal perforation and pe ritonitis may be

accompanied by:
 a sudden rise in pulse rate,
 hypotension,
 marked abdominal tenderness and guarding, and
 subsequent abdominal rigidity.
238
 A rising white blood cell count with a left shift and free air on
abdominal radiographs may be seen in such cases.

Refere nces
1. Southall D, Coulter B, Ronald C, International Child Health
Care, A practical manual for hospitals worldwide pg 426-428
2. Kliegman, Behrman, Jenson, Stanton, Nelson Textbook of
Paediatrics, 19th Edition, 2007

SCHISTOSOMIASIS

Definition
Schistosomiasis is an acute and chronic parasitic disease caused
by blood flukes (trematode worms) of the genus Schistosoma.

Causes
 People become infected when larval forms of the parasite
released by freshwater snails penetrate the skin during
contact with infested water
 There are 2 major forms of schistosomiasis that are caused by
5 main species of blood fluke:

I. Intestinal
o Schistosoma mansoni,
o Schistosoma japonicum
o Schistosoma mekongi (uncommon)
o Schistosoma intercalatum (uncommon)

II. Urogenital

o Schistosoma haematobium

Clinical Features
In the acute infection, mild, maculopapular skin lesions may
develop within hours after exposure. Depending on which
species is responsible for the infection, the clinical features will be
distinguished between intestinal, urogenital or both:

239
 Intestinal schistosomiasis
o Acute: Abdominal pain, diarrhoea, blood in the stool,
fatigue.
o Chronic: hepatomegaly, splenomegaly ascites, portal
and pulmonary hypertension.

 Urogenital Schistosomiasis
o Acute: Haematuria, dysuria, urinary frequency.
o Chronic: Fibrosis of the bladder and ureter, kidney
dysfunction, bladder cancer (later stages).
o Adolescent girls: genital lesions, vaginal bleeding, pain
during sexual intercourse, nodules in the vulva.
o Adolescent boys: pathology of the seminal vesicles,
prostate and other organs.

Investigations
 Stool/Urine M/C/S for blood (including occult) and
Schistosoma ova.
 FBC, DC (usually shows eosinophilia).
 Urea, Electrolytes, LFTs.
 Chest x-ray
 Plain abdominal x rays
 Abdominal ultrasound
 Blood culture (if possible concomitant disease i.e.
salmonellosis).
 Rectal Snip

Treatme nt
 Praziquantal is the treatment of choice for all forms of
Schistosomiasis (children >2 years and adults: 40mg/kg as a
single dose). Consider steroid therapy if very severe disease.
 Adverse effects of praziquantel include dizziness, headache,
nausea, vomiting, diarrhea, abdominal discomfort, bloody
stool, urticaria, and fever following initiation of treatment.
These are usually mild and last about 24 hours.

Prevent ion

240
Praziquantel 40/mg/kg as a single dose.

Refere nces
1. Medicins sans frontieres, Clinical Guidelines
2. WHO, Fact sheet, Schistosomiasis, 2019
3. Shadab Hussain Ahmed, Schistosomiasis (Bilharzia), 2018,
Medscape

4. Nelson’s Textbook of Pediatrics, 19th edition

241
TUBERCULOSIS IN CHILDREN
TB in children is an indicator of recent and ongoing transmission
of M. tuberculosis in the community, as majority of children will
develop tuberculosis disease within 1 year after infection. (Unlike
tuberculosis in adults who may develop TB disease when their
immunity goes down).
Pulmonary TB is the commonest type of TB in children but
extrapulmonary disease is also common estimated to be around
30-40% of cases.

Most immunocompetent children with TB disease present with

nonspecific symptoms of a chronic disease. In infants the
presentation may be more acute and can present as acute
severe, recurrent or persistent pneumonia. TB should be
suspected when there is a poor response to appropriate
conventional antibiotics.

Key risk factors for TB in c hildren

 Household contact with a newly diagnosed smear positive
case.
 Age < 5 years
 HIV infection
 Severe malnutrition

Symptoms of tuberculo sis

TB should be suspected in children presenting with following
symptoms especially if they persist for more than 2 weeks or show
no response to (appropriate) treatment for the initial diagnoses.
 Cough
 Fever
 Loss of appetite
 Weight loss or failure to thrive
 Decreased activity.

Other symptoms will depend on the anatomical site of

tuberculosis disease.

Clinical signs suggestive of pulmo nary TB.

242
There are no specific features on clinical examination that can
confirm that the presenting illness is due to pulmonary TB.

Clinical signs suggestive of ext rapulmo nary TB.

 Gibbus- Spinal TB, especially of recent onset.
 Non-painful enlarged cervical lymphadenopathy, with or
without fistula formation- TB of lymphnodes.
 Pleural effusion
 Pericardial effusion
 Distended abdomen with ascites- TB abdomen.
 Non painful enlarged joints- Osteoarticular TB.
 Meningitis not responding to antibiotic treatment - TB
meningitis.

Guidance on approac h to diagnosis of TB in childre n

 Careful history (including history of TB contact and symptoms
consistent with TB).
 Clinical examination (including growth assessment).
 Bacteriological confirmation with Xpert MTB/RIF or culture or
smear microscopy).
 Chest X-ray
 Tuberculin skin testing
 Urinary lateral flow lipoarabinomannan (LAM) for HIV infected
children or presumed severe TB disease.
 Investigations relevant for suspected extra-pulmonary TB.
 HIV testing

Appropriate specimens should be obtained for Xpert MTB/RIF

testing, staining and microscopy, culture (and histopathological
examination in extrapulmonary TB).
For pulmonary tuberculosis

Samples for Xpert MTB/RIF or culture testing should be obtained

using any the following methods depending on the age of the
child;
 Gastric lavage
243
 Sputum induction
 Nasopharyngeal aspiration
 Expectorated sputum

For extrapulmonary TB

 Pleural fluid: Xpert MTB/RIF, biochemistry, cell count and

culture.
 Pericardial fluid: Xpert MTB/RIF, biochemistry, cell count and
culture.
 Lymph node biopsy or Fine-needle aspiration of enlarged
lymph glands: Xpert MTB/RIF, ZN staining, culture and
histology.
 CSF: Biochemistry, cell count, Xpert MTB/RIF.
 In addition, appropriate imaging studies such as abdominal
ultrasound, Echocardiogram, CT and MRI should be used
depending of the site of disease of extrapulmonary disease.

Recommended TB treatment regimens in c hildren

Recommended regimen
TB disease category
Intensive phase Continuation
phase
All non-severe forms of PTB and 2 (HRZE) 4 (HR)
EPTB
Severe forms - TB meningitis, 2 (HRZE) 10 (HR)
Osteo-articular TB, Spinal TB, Miliary
TB, other severe forms of TB

TB drug dosing t able

Number of Tablets
Intensive Phase Continuation
Phase
Weight bands RHZ (75/ 50/150 mg) E* (100 mg) RH (75/50 mg)
4-7 kg 1 1 1
8-11kg 2 2 2
12 – 15 kg 3 3 3

244
 16- 24 kg 4 4 4
25 and above

Rifampicin (R) – 15 mg/kg (range 10 – 20 mg/kg); maximum dose

600 mg/kg, Isoniazid (H) –10 mg/kg (range 7 – 15 mg/kg);
maximum dose 300 mg/kg, Pyrazinamide (Z) – 35 mg/kg (30 – 40
mg/kg) and Ethambutol (E) – 20 mg/kg (15 – 25 mg/kg)

Use of corticosteroids
Corticosteroids are indicated in the management of some
complicated forms of TB such as:
 TB meningitis.
 Complications of airway obstruction by TB lymph glands.
 Pericardial TB.
Prednisolone is recommended at a dose of 2mg/kg daily,
increased to 4 mg/kg daily in the case of the most seriously ill
children, with a maximum dosage of 60 mg/day for 4 weeks. The
dose should then be gradually tapered over 1–2 weeks before
stopping.

Pyrido xine supplement ation.

Isoniazid may cause symptomatic pyridoxine deficiency, which
presents as neuropathy, particularly in severely malnourished
children and HIV-positive children on antiretroviral therapy (ART).
Supplemental pyridoxine (5–10 mg/day) is recommended in HIV-
positive or malnourished children being treated for TB.

Drug resistant tuberculosis in childre n

Drug-resistant TB (DR TB) should be suspected under the following
conditions:

 Close contact with a person known to have DR-TB including

household and other contacts.
 Close contact with patient that died from TB, failed or is not
adherent to TB treatment.
 History of previous TB treatment (in the past 6-12 months).
 Not improving after 2-3 months of first line TB treatment,
including persistence of positive smear or culture, persistence

245
 of symptoms and failure to gain weight (radiological
improvement is frequently delayed).
 A child who develops active TB while on Isoniazid (INH)
prophylaxis.
Appropriate samples should be obtained in any suspected case
of DR-TB. These should include the following:
 Xpert MTB-RIF
 Mycobacterial culture
 drug susceptibility testing (DST)
 line-probe assays (LPA)

Indications to initiate MDR TB re gimen in children;

 Confirmed MDR TB by DST or LPA.
 RR on Xpert MTB/RIF.
 Smear positive case with confirmed MDR contact.
 Child with TB and unconfirmed DST resulting who is not
responding to standard TB therapy and is a known contact of
an MDR TB case.

For treatment of DR TB refer to the Guidelines for the

Programmatic Management of Drug Resistant Tuberculosis.

Indications for Tuberculosis preventive therapy (TBT) in children

TB preventive therapy is recommended for the following at risk
groups of children
 Asymptomatic HIV negative children aged <5 years who are
household contacts of bacteriologically confirmed
tuberculosis in whom active TB disease has been excluded.
 All HIV infected children aged, regardless of TB contact status,
in who are considered unlikely to have active TB disease. For
HIV infected infants preventive therapy is provided only when
child has established TB contact.

Recommended and dosing options fo r TBT in children

Drug regimen Dose per kg body weight Maximum dose
Isoniazid daily for 6 – 9 H 10 mg (range, 7 – 15 mg) 300 mg
months

246
 isoniazid plus rifampicin H 10 mg (range, 7 – 15 mg) H – 300 mg
daily for 3 – 4 months
R 15 mg (range, 10 – 20 mg) R – 600 mg
Rifapentine plus H: Age ≥ 12 years: 15 mg/kg, H 900 mg
isoniazid Weekly for 3
H: Age 2 – 11 years: H 25 mg/kg
months (12 doses)
Rifapentine, 900
Rifapentine mg
10.0 – 14.0 kg = 300 mg
14.1 – 25.0 kg = 450 mg
25.1 – 32.0 kg = 600 mg
32.1 – 49.9 kg = 750 mg
≥ 50.0 kg = 900mg

Refere nces
1. Desk guide for the management of tuberculosis in children-
Africa. 3rd Edition. International Union Against TB and Lung
Disease. 2016.
2. National Tuberculosis and Leprosy Programme-Guidelines for
the management of tuberculosis in children. 1st Edition. 2016.
3. National Tuberculosis and Leprosy Programme-TB Manual. 5 th
Edition. 2017 Guidance for national tuberculosis programmes
on the management of tuberculosis in children. WHO 2014.
4. Guidelines for the programmatic management of drug-
resistant tuberculosis in Zambia. 3rd Edition. 2018.
5. Guidelines for the management of latent tuberculosis
infection. 2nd edition. 2019.

247
POST EXPOSURE PROPHYLAXIS - OCCUPATIONAL HIV
EXPOSURE
Post exposure prophylaxis is the use of ART to prevent HIV
transmission, in adults exposure occurs commonly from
occupational injuries.
The risk of infection after occupational exposure to HIV infected
blood is low and estimated at:
 1: 300 in percutaneous exposure
 1: 1000 in mucocutaneous exposure

There is no risk of transmission if the skin is intact but there are

factors that can increase the risk of infection e.g.
 Deep injury
 Visible blood on device caused injury
 Injury with a large bore needle
 Non-suppressed viral load in source patient

Other body fluids that can lead to HIV transmission include:

amniotic fluid, CSF, breast milk, pericardial, pleural, peritoneal
fluids, synovial fluid, unfixed human tissue, vaginal secretions,
semen, any other visibly blood stained fluid, fluid from burns or
skin lesions.

Management o f occ upational exposure

Immediately after exposure to infectious .
 Clean site: wash skin injuries with soap and running water, do
not squeeze, allow wound to freely bleed.
 If the exposed area is eye or mucous membrane, rinse with
copious amount of clean water.
 Contact the person responsible for PEP/ Medical staff on duty.
 Person in charge of PEP should assess need for PEP and
benefits of taking ART prophylaxis.

248
PEP Tre atment Regimen
Risk Category ART prophylaxis Duration
Skin intact No PEP
Medium Risk- invasive injury, no Preferred: TDF +XFTC + DTG
visible blood on Alternative: TDF + XTC + ATV/r
needle/device
Children below 10 yrs: AZT 28 days
High Risk-large volume of +3TC+ LPV/r
blood/fluid, deep extensive
injury, known HIV infected
patient, large bore needle

ELIMINATION OF MOTHER TO CHILD HIV TRANSMISSION -

EMTCT
Four (4 ) P illars of EMTCT
3. Primary prevention of HIV infection in women of child
bearing age- IEC-STIs , HIV, provision of condoms, PrEP for
discordant couples, ART for positive partners.
4. Prevention of unwanted pregnancies among women living
with HIV: Counselling and provision of Family planning
services.
5. Prevention of HIV transmission in a woman living with HIV to
her infant: Testing and ART initiation in all HIV positive
pregnant and breastfeeding mothers.
6. Provision of appropriate treatment, care and support to
women and children with HIV and their families. Treatment
and adherence support for HIV positive women and
infected infants, ART prophylaxis for their uninfected infants.

Management o f HIV exposed uninfecte d infants

(HEU)
Situation Management of Infant prophylaxis, and
mother at delivery Tests
and Postnatal care
High Risk HIV exposed Infant

249
 HIV +Mother less 12 Start or continue Start ART prophylaxis - AZT
weeks on ART ART +3TC+NVP for 12 weeks
HIV + mother, viral
load > 1000copies
within 4 weeks before
delivery
HIV + ve mother not Start ART or Start ART prophylaxis
on ART continue ART AZT+3TC+NVP - ,continue
HIV +ve mother who untill confirmed HIV
Counselling for negative after cessation
refuses ART
mother to start ART of breastfeeding

Low Risk HIV exposed infants

HIV +ve mother, on Continue ART Start ART- AZT+3TC+NVP
ART for > 12 weeks for 6 weeks
HIV -ve woman with NAT, if negative- put if mother’s NAT test is
an HIV+ve partner woman on PrEP, +ve, NAT on infant,
repeat HIV test
every 3 months

NOTE: All HIV positive pregnant women should have

viral load testi ng within 4 weeks before delivery.

Dosing table for the EMTCT AR T Prophylaxis

Age of Infant
Dose from Birth to 6 weeks 6 weeks to 12
weeks
Weight 2000gm-2499gm 2500gm-2999gm 3000gm-5990gms

AZT+ 3TC 10mg/5mg 15/7.5mg twice 60/30 mg tablet

Suspension twice daily daily 1 tab twice daily
*1ml twice daily 1.5 mls twice daily

NVP 10mg once daily 15mg once daily 20mg once daily
1ml syrup once 1,5 mls syrup Or 1/2 tablet of
daily 100mg tablet
once daily

AZT +3TC is a tablet co ntaining 60mg AZT and 30mg

3TC

250
 Dissolve 1 tablet of AZT+3TC in 6mlf of water, 1ml of suspension
has 10mg of AZT and 5mg of 3TC
 The suspension made should be kept in a cool place and
used as soon as possible. Daly reconstitution is recommended.

Follow up of HEU infant, breastfeeding, non

breastfeeding
In an HEU infant who is breastfed

NAT at birth/first week of life or first contact with health staff, at 6

weeks, at 9 months and 6weeks after cessation of breast
feeding.

251
ENDOCRINE

DISORDER OF SEXUAL DIFFERENTIATION (DSD)

Definition
 DSDs include anomalies of sex chromosomes, gonads,
reproductive ducts and the genital.
 Usually presents in the neonatal period and during
adolescence
 Neonates usually present with atypical genitalia while
adolescents present with atypical sexual development during
puberty

Causes
1. 46 XY DSD
o 46XY gonadal dysgenesis (partial or complete)
o 46XX/46XY (chimeric, ovotesticular DSD)
o Testicular regression syndrome
2. 46XX DSD
o 46 XX gonadal dysgenesis
o 46XX ovotesticular DSD
o 46 XX testicular syndrome
3. Disorders in sex development due to androgen excess (CAH)
o 21 hydroxylase deficiency
o 11B hydroxylase deficiency
o 3b HSD deficiency
4. Disorders of sexual development due defective hormone
synthesis or action
o Androgen insensitivity syndrome (AIS)—can be partial
(PAIS) or complete (CAIS
o 5-alpha reductase deficiency (5-AR deficiency)
o LH receptor defect
5. Others
o Maternal hyper-androgen exposure

252
 o Placental aromatase deficiency

Clinical features
 Ambiguous genitalia
 Bilateral cryptorchidism
 Hypospadias
 Micropenis
 Delayed puberty
 Atypical puberty

Diagnostic approac h to a pat ient with DSD

Ambiguous genitalia/pubertal problems

Karyotype 46,XY

Absent Gonads Testis-Testis

Uterine ± Ovotestis Disgenetic testis Streak-Streak

Agonadism Uterine ± Uterine ± Uterine + Uterine -

Ovotesticular Partial Complete

DSD
Disorder Androgen
Gonadal Gonadal
Synthesis or Action
Dysgenesis Dysgenesis
Testerone Biosynthetic
Defect
LH-Receptor mutation
5-Reductase Deficiency
POR Gene Defect
Timing Defect

Figure XXXa. Diagnostic Algorithm of 46,XY DSD for new classification.

DSD. Disorders of sexual development

253
 Ambiguous genitalia/pubertal problems

Karyotype 46,XX

Gonads

Ovary-Ovary Ovotestis Streak-Streak Testis-Testis

Uterine + Uterine + Uterine + Uterine -

Gonadal
Virilized female Ovotesticular DSD
Dysgenesis XX Testicular DSD

17OH-Progesterone
Normal
High

CAH NON-CAH

Figure XXXb. Diagnostic Algorithm of 46,XY DSD for new classification.

DSD. Disorders of sexual development

Treatme nt
Treatment of a patient with DSD aims to;
 Confirm the diagnosis
 Promptly rule out life threatening emergency (CAH or
hypopituitarism)
 Sex assignment

Management of a patient with DSD should be family/patient

centered with a multi-disciplinary approach:

254
 Rule out CAH in every child with genital ambiguity (if patient in
adrenal crisis treat the shock, glucocorticoids and
mineralcortocoids)
 Sex assignment is a multi-step process and should be done by
a multidisciplinary team with the involvement of the patient
 A multi-disciplinary team should comprise of paediatrician,
paediatric endocrinologist, paediatric surgeon, urologist,
psychologist and social worker
 Continuous counselling and psychological care should be
offered to the family through out the process of care.

Import ant notes:

 DO NOT assign sex at birth to a child with atypical genitalia. If
the child has to be named advise the parents to assign a sex
neutral name and DO NOT call the child, he or she until
gender is appropriately assigned
 The parents should be reassured in an empathetic manner
that the child’s sex shall be determined collectively after
appropriate investigations are conducted
 A child with atypical genitalia must be referred to a centre
with a paediatrician for appropriate management

Refere nces
1. Henriette A Delemarre-van de Waal and Peggy T Cohen-
Kettenis; Clinical management of gender identity disorder in
adolescents: a protocol on psychological and paediatric
endocrinology aspects: European Journal of Endocrinology
(2006) 155 S131–S137
2. Mariana Telles-Silveira, Felicia Knobloch; Management
framework paradigms for disorders of sex development:
Arch Endocrinol Metab. 2015;59/5
3. S. Faisal Ahmed*, John C. Achermann Society for
Endocrinology UK guidance on the initial evaluation of an
infant or an adolescent with a suspected disorder of sex
development (Revised 2015): Clinical Endocrinology (2016)
84, 771–788
4. Ganka Douglas, Marni E. Axelrad; Review Article ,Consensus
in Guidelines for Evaluation of DSD by the Texas Children’s
Hospital Multidisciplinary Gender Medicine Team:

255
 International Journal of Pediatric Endocrinology Volume
2010, Article ID 919707, 17 pages doi:10.1155/2010/919707
5. Mimi S. Kim, MD, Anna Ryabets-Lienhard, Mitchell E. Geffner,
MD; Management of Congenital Adrenal Hyperplasia in
Childhood: Curr Opin Endocrinol Diabetes Obes . 2012
December ; 19(6): 483–488. doi:10.1097/MED.
0b013e32835a1a1b.
6. Aimee M. Rolston, Melissa Gardner; Disorders of Sex
Development (DSD): Clinical Service Delivery in the United
States: Am J Med Genet C Semin Med Genet . 2017 June ;
175(2): 268–278. doi:10.1002/ajmg.c.31558.

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RICKETS

Definition
Rickets is a disease due to defective mineralization at growth
plates in growing bones.

Aetiolo gical classificat ion

Calcipenic rickets; inadequate Nutritional vitamin D deficiency
vitamin D, defective utilization of Calcium deficiency
vitamin D or inadequate calcium
Distal renal tubular acidosis
Phosphopenic rickets; is not common Low phosphorus intake
but occurs in special situations Renal phosphate wasting
Prematurity/ total parenteral
nutrition

Symptoms and signs

Age category Signs and symptoms

During infancy  Delayed milestones

 Delayed dentition
 Craniotabes
 Irritability
 Sweating
 Delayed closure of anterior fontanelle
 Bossing of the skull
Older children  Waddling gait,
 Harrison sulcus,
 Rachitic rosary,
 Genu valgum,
 Genu varum (intercondylar distance more
than 5cm) or windswept deformity are known
to occur
Adolescents  Seizures and bone pain are the major features
 Short stature may be a feature in
hypophosphataemic rickets.

Investigations

257
Biochemical investigations

 U&Es, Creatinine
 FBC
 Bone profile (Ca, Mg, phosphate, alkaline phosphatase)
 25-OH Vitamin D levels (combined vitamin D2 and D3)
 Additional testing may be required depending on context

Radiological investigat ion

 X- rays of wrist and knee

Classificatio n for serum 25(OH )D;

 Sufficiency: >50 nmol/L (>20 ng/mL);
 Insufficiency: 30–50 nmol/L (12–20 ng/mL);
 Deficiency: <30 nmol/L (<12 ng/mL).

Definite rickets  Rachitic changes on radiographs (cupping

and fraying of metaphysis, widening of
epiphyseal plate)
 High blood alkaline phosphatase
 Hypophosphataemia or hypocalcaemia
 Clinical signs: bone deformities such as genu
varum and valgum, abnormal spinal
curvature, craniotabes, open fontanelles,
rachitic rosary, joint swelling
Possible rickets  Rachitic changes on radiographs (cupping
and fraying of metaphysis, widening of
epiphyseal plate)
 High blood alkaline phosphatase
 Hypophosphataemia hypocalcaemia

Treatme nt
Age Vit D daily dose Vit D single Vit D Calcium
for 12 weeks dose maintenance dose
dose (IU) mg/day
<3 mo 2000 IU/day N/A 400 500
3 -12 mo 2000 IU/day 50,000 400 500

258
 >12 mo – 3000–6000 150,000 600 1000
12 yr IU/day
>12 yr 6000 IU/day 300,000 600 1000

Import ant Notes

 Maintenance dose to be given until resolution of symptoms
 Patients require monitoring of nutritional rickets after the onset
of treatment until resolution of symptoms
 Patients with non-nutritional rickets should be managed at a
tertiary centre
 In children with tetany or convulsions, treat with intravenous
calcium gluconate at a rate of 10–20 mg/kg in 5–10 minutes,
repeated as needed. (for calcium treatment in severe
hypocalcaemia refer to hypocalcaemia protocol)

Refere nces
1. Anuradha Khadilkar, Vaman Khadilkar, Prevention and
Treatment of Vitamin D and Calcium Deficiency in Children
and Adolescents: Indian Academy of Pediatrics (IAP)
Guidelines
2. Navoda Atapattu, Approach to a child presenting with
rickets;Sri Lanka Journal of Child Health, 2013; 42(1): 40-44
3. Nutritional rickets, a review of disease burden, causes,
diagnosis, prevention and treatment; WHO 2019
4. Ji Yeon Lee, Tsz-Yin So, and Jennifer Thackray; A Review on
Vitamin D Deficiency Treatment in Pediatric Patients, J
Pediatr Pharmacol Ther 2013;18(4):277–291
5. Vitamin D Deficiency and Nutritional Rickets:
Supplementation and Treatment in Infants and Children

259
PRECOCIOUS PUBERTY (PP)

Introduction
Puberty is the period of physical, hormonal and psychological
transition from childhood to adulthood, with accelerated linear
growth and achievement of reproductive function. Puberty starts
at age 8 in females and 9 in males.

Definition
Precocious puberty is defined as onset of puberty before age 8
in girls and before age 9 in boys.

Classificatio n of Precocious pube rty

Precocious puberty is classified according to the underlying
physiopathologic mechanisms as;
 Variants of normal pubertal development; isolated forms of
thelarche, pubarche and vaginal bleeding which may or may
not be of hormonal aetiology
 Central precocious puberty (CPP); gonadotropin dependent-
precocious puberty also called true precocious puberty. Due
to early maturation of the HPG axis
 Peripheral precocious puberty; gonadotropin-independent
precocious puberty also called pseudo-precocious puberty

Aetiolo gy
 Central precocious puberty(CPP); causes include; idiopathic,
genetic (e.g. activating mutation of KISS1R and KISS1 gene),
hypothalamic hamartomas, tumours (e.g.
craniopharyngioma, astrocytoma, ependymoma,
neurofibroma e.t.c.), cerebral malformations (e.g.
hydrocephalus, spina bifida, meningomyelocele, septo-optic
dysplasia, vascular malformations), acquired disease such as
meningitis, sarcoidosis, tuberculous meningitis, radiation
 Peripheral precocious puberty; McCune Albright Syndrome
(MAS), ovarian cyst, oestrogen secreting ovarian tumours,
oestrogen secreting adrenal tumours, exogenous oestrogen
exposure, CAH, Leydig cell tumours, exogenous exposure to
testosterone

260
Symptoms and signs

The clinical manifestation of puberty include the following;

 Girls; breast development (thelarche), pubic hair (pubarche),
axillary hair, apocrine ador, menarche, eostrogenization of
vaginal epithelium
 Boys; testicular enlargement, penile enlargement, pubic hair
development, axillary hair, breaking of voice
 Accelerated growth in both sexes

Evaluation of the patient with precocious puberty

 Onset and progression of symptoms
 Presence of neurological symptoms
 Current and previous therapies (e.g. radiation therapy)
 Height, weight, BMI (plotted on growth charts)
 Height velocity
 Target height

Diagnostic testing
 Height, weight, BMI plotted on appropriate growth charts
 Height velocity
 X-ray of left wrist and hand for bone age determination
 Pelvic ultrasound
 Additional testing may include; CT scan or MRI, testing for
underlying condition as guided by history and physical
examination
 Hormonal tests (LH, FSH, oestrogen, testosterone, GnRH
stimulation test)

Treatme nt
Treatment of precocious puberty depends on the type and
cause.
Isolated benign variant of puberty;

261
 Premature thelarche, vaginal bleeding, premature pubarche,
lipomastia without any other sign of puberty usually just
requires surveillance every three to six months
 Parents need adequate counselling to alley anxiety
Peripheral precocious puberty

 Treatment is based on the cause (e.g. MAS, exogenous

steroids, gonadal tumours etc)
Central precocious puberty

 The goal of therapy is preservation of adult height potential

and manage psychological difficulties that come with early
puberty
 The main stay of treatment is GnRHa e.g. leuprolide acetate.
 Administered every 4 weeks
 Monitor height velocity, breast and testicular size
 Dosage is age dependant

Body Weight Recommended Dose

≤ 25 kg 7.5 mg
> 25-37.5 kg 11.25 mg
> 37.5 kg 15 mg

Referral crite ria for a patient with PP

 Every patient with suspicion of PP should be referred to a
second or third level hospital with a presence of a
paediatrician
 Patients with PP should be managed by a paediatrician or
paediatric endocrinologist

262
Precocious Puberty e valuatio n flow chart

Refere nces
1. D Mul and A Hughes; The use of GnRH agonist in precocious
puberty; European Journal of Endocrinology (2008) 159 S3–
S8,

2. V N Brito, A M Spinola-Castro; Central precocious puberty:

revisiting the diagnosis and therapeutic management; Arch
Endocrinol Metab. 2016;60(2):163-72, DOI: 10.1590/2359-
3997000000144
263
3. John S. Fuqua; Treatment and Outcomes of Precocious
Puberty: An Update. JClinEndocrinolMetab98: 2198–2207,
2013)
4. David A. Klein, Jill E. Emerick, Disorders of Puberty: An
Approach to Diagnosis and Management: American
Family Physician, www.aafp.org/afp@2017
5. Paul Kaplowitz, Clifford Bloch; Evaluation and Referral of
Children With Signs of Early Puberty: PEDIATRICS Volume 137,
number 1, January 2016

264
HYPOGLYCAEMIA IN CHILDREN AND ADOLESCENTS

Definition
Hypoglycaemia is defined as plasma glucose of less than 2.6
mmol/l. However, of note is that in preterm neonates in the 1 st
three days of life, glucose maybe as low as 1.1 mmol/l without
any underlying abnormality. In term neonates, it may be as low
as 1.7 mmol/L in the first three days and 2.2 mmol/l in the
remainder of the week. Thereafter, a glucose of 2.6 mmol/L or
lower requires investigations.

Causes
 Intrauterine growth retardation and prematurity
 Perinatal asphyxia
 Infant of a diabetic mother
 Intrauterine infections and sepsis
 Rhesus incompatibility
 Inborn errors of metabolism
o Amino acids and organic acids
o Disorders of carbohydrate metabolism e.g. glycogen
storage disease, fructose intolerance, lactosaemia
o Fatty acid oxidation defects
o Urea cycle defects
 Endocrine causes
o Hypopituitarism
o Growth hormone or adrenal insufficiency
o Persistent hyperinsulinaemic hypoglycaemia of infancy
o Beckwith-wiedemann syndrome
o Insulinoma
 Ketotic hypoglycaemia
 Drugs including alcohol, aspirin, beta blockers
 Sepsis especially due to gram negative organisms

265
Signs and symptoms
Hypoglycaemia is often accompanied by signs and symptoms
of autonomic (adrenergic) activation and/ or neurological
dysfunction (neuroglycopenia)
 Autonomic signs and symptoms
o Tremors
o Pounding heart
o Cold sweatiness
o Pallor
 Neuroglycopenic signs and symptoms
o Difficulty concentrating
o Blurred vision
o Disturbed colour vision
o Difficulty hearing
o Slurred speech
o Poor judgment and confusion
 Problems with short-term memory
 Dizziness and unsteady gait
 Loss of consciousness
 Seizure
 Death
 Behavioral signs and symptoms
o Irritability
o Erratic behavior
o Nightmares
o Inconsolable crying
 Non-specific symptoms (associated with low, high or normal
blood glucose)
o Hunger
o Headache
o Nausea

266
 o Tiredness

Investigation of hypoglycaemia
Blood taken for a diagnostic screen is only useful if taken when
the patient is hypoglycaemic (glucose < 2.6 mmol) and should
include the following:
 Glucose
 Insulin
 Cortisol
 Growth hormone
 Lactate
 Free fatty acids
 Amino acids
 Ketone bodies ( hydroxyl butyrate and acetoacetate)
 Urine
 Organic acids

NOTE: In hypoglycaemic states and in the absence of ketones, it

is important to look at free fatty acids (FFA). Normal FFA suggests
hyperinsulinism and raised FFA suggests a fatty acid oxidation
defect. Hypoglycaemia in the presence of urinary ketones
suggests either a counter regulatory hormone deficiency or an
enzyme defect in the glycogenolysis or gluconeogenesis
pathways.

Treatme nt
Hypoglycaemia should be treated promptly after obtaining the
critical intravenous samples by intravenous infusion of 5 ml/kg of
10% dextrose followed by adequate glucose infusion to maintain
euglycaemia.

Severe hypoglycemia can be reversed by the injection of

glucagon: glucagon 0.5 mg < 12yr, 1.0 mg for ages >12yr or 10-
30 mcg/kg body weight. Glucagon is given intramuscularly or
subcutaneously.

267
Treatment should also be aimed at the underlying cause of
hypoglycaemia. Fig XX shows flow chart for the emergency
management of hypoglycaemia

268
 DO NOT give glucose immediately. Take a second and think about it:
Is the blood glucose less than 45 mg/dL?

Does the patient have a known cause? (e.g. insulin, sulfonylurea)

YES
NO
Patient Alert?
NO
YES Meets 1st 2 criteria of Whipple’s Triad
Signs or sumptoms of hypoglycaemia
Treatment Blood glucose <45 mg/dL
(Symptoms resolve with glucose
 Oral glucose 15g (e.g. 4 oz OJ) intake)
 Check glucose in 10 – 15 min
prn
 Repeat glucose prn
Draw critical blood samples
 5 – 10 mL for serum (SST)
Treatment o Glucose
o Insulin
 IV glucose 0.25 g/kg bolus OR
o Cortisol
 IM glucagon 0.5 – 1.0 mg o Growth hormone
 Check glucose in 10 – 15 min prn o Beta hydroxybutyrate
 (Sooner if no clinical response) o ± Plasma amino acids
 Maintain IV glucose infusion prn o ± Carnitine
o ± Lactate/pyruvate on ice

Next urine
Look for cause (± admission)
o Dip for ketones
 Age
o Organic acids
 Last meal o Toxicology (ASA, ethanol,
 Severe hypoglycaemia sulfonylureas)
o Accidental vs intentional o NOT routine screen
o High activity
o Low food intake
o Change in normal routine/caretaker
o Illness Preventive Recurrence
o Medication dose change
 Education
 Know metabolic disorder  Start of change current treatment
 Drugs  Follow-up

Figure X Emergency management of hypoglycaemia

269
Refere nces
1. Clarke W et al. Assessment and management of
hypoglycemia in children and adolescents with diabetes.
Pediatric diabetes 2008: 9:165-174
2. Cox DJ et al. Perceived symptoms in the recognition of
hypoglycemia. Diabetes care 1993:16:519-527

3. Krishna MG, Gupta DK. Hutchison Paediatrics: Pages 437-438

4. Kliegman RM et al. Nelson Textbook of Pediatrics: Pages 655-
668

270
GRAVE’S DISEASE (GD)

Introduction
Graves disease is the most common form of hyperthyroidism in
children, due to an autoimmune induced overproduction of
thyroid hormones. In Graves disease the immune system
produces antibodies called thyroid stimulating immunoglobulins
(TSIs) that bind to thyroid receptors and activate production of
thyroid hormones outside of the negative feedback mechanism
that occurs with normal thyroid function. GD may be associated
with other autoimmune diseases such as type 1 diabetes mellitus,
vitiligo, rheumatoid arthritis etc.

Symptoms and signs

Common Symptoms of GD include:
 Heat intolerance, sweating
 Palpitation
 Exophthalmos
 Nervousness
 Irritability
 Emotional instability
 Tremor
 Weight loss in spite of increased appetite,
 Deterioration of attention,
 Restless sleep,
 Fatigue
 Nocturia, frequent micturition,
 Irregular menstrual periods

Signs
 Tachycardia, atrial fibrillation
 Goiter
 Ophthalmopathy
 Diffuse goiter
 Wide pulse pressure,

271
 Uncoordinated movements,
 Pretibial myxedema
 Anxiety
 Muscle wasting
 Signs of heart failure

Labo ratory test Investigations

Type of test Values
TSH 0 – 1uU/ml in severe disease (1 -3
uU/ml in mild disease
FT4
FT3

Thyroid ultrasound scan; is useful to confirm or exclude the

presence of a nodule particularly if a colour doppler if
performed
Antithyroid antibodies; the presence of antibodies provide
supporting evidence for Graves disease.

 More than 95% of patients with GD are positive for TPO

(thyroperoxidase antibody)
 About 50% have positive anti-thyroglobulin antibody titres
 Thyroid stimulating-thyrotropin receptor antibodies (TRab, TSI)
present in about 90%
Radioisotope scan is of little value in the diagnosis of graves
disease

Treatme nt
Antithyroid drug therapy
 Carbimazole 0.5-1 mg/kg
 Propylthiouracil (PTU) 5-10 mg/kg

Iodine-131 therapy

272
 Radioiodine ablation of the thyroid gland is quick, easy,
moderatly expensive, avoids surgery, and is without significant
risk in adults and probably teenagers.

Surgery
 Thyroidectomy was the main therapy, has been to a large
extent replaced by 131-I treatment.

Refere nces
1. Helena Jastrzębska (2015)Antithyroid drugs; Thyroid Research
volume 8, Article number: A12 (2015) 7164 Accesses

2. Kanshi Minamitani, Hirokazu Sato, Hidemi Ohye, Shohei

Harada, and Osamu Arisaka (2017); Guidelines for the
treatment of childhood-onset Graves’ disease in Japan, Clin
Pediatric Endocrinology 2017; 26(2), 29–62 Copyright© 2017
by The Japanese Society for Pediatric Endocrinology,

3. Leslie J DeGroot, (2016), Diagnosis and Treatment of Graves’

Disease, Endotext
4. Wayne EJ: The diagnosis of thyrotoxicosis. Br Med J 1:411,
1954.

5. Sawin CT, Geller A, Kaplan MM, Bacharach P, Wilson PWF,

Hershman JM. Low serum thyrotropin (thyroid stimulating
hormone) in older persons without hyperthyroidism. Arch
Intern Med 151:165-168, 1991.

6. Brown ME, Refetoff S: Transient elevation of serum thyroid

hormone concentration after initiation of replacement
therapy in myxedema. Ann Intern Med 92:491, 1980.

273
HYPOTHYROIDISM

Introduction
Neonatal hypothyroidism results from decreased T4 production in
a newborn. It is the most preventable cause of potential
intellectual disability.
Childhood hypothyroidism also known as acquired
hypothyroidism usually occurs after 6 months of age. The
hypothyroidism is caused by failure of the hypothalamic-
pituitary-thyroid axis, which results in decreased production of
thyroid hormones. The hypothyroidism may be primary (at the
level of thyroid gland), secondary (at the level of pituitary
gland), or tertiary (at the level of hypothalamus).

Symptoms and Signs

Congenital
 More than 95% of newborns who have congenital
hypothyroidism have little if any symptoms or signs at birth.
 Some maternal T4 crosses the placenta, so infants who cannot
make any thyroid hormone still have serum T4 concentrations
that are 25% to 50% of normal.
 Birth length and weight are within the normal range, but head
circumference may be increased.
 An open posterior fontanelle in a term baby may signal
congenital hypothyroidism.
 Lethargy
 Hypotonia
 Hoarse cry
 Feeding problems
 Constipation
 Macroglossia
 Umbilical hernia
 Dry skin
 Hypothermia, and prolonged jaundice
 Goiter is uncommon

274
 Newborns who have congenital thyroid dyshormonogenesis
may have a palpable goiter, but the goiter typically develops
later in untreated patients.

Acquired
 Decline in linear growth
 Fatigue
 Constipation
 Cold intolerance
 Poor school performance
 Weight gain
 Irregular menstrual periods, and somnolence
 Children afflicted with Hashimoto thyroiditis also may have
other autoimmune disorders and a family history of thyroid
and other autoimmune disorders to support the diagnosis.
 Other clinical features of acquired hypothyroidism include
bradycardia
 Short stature
 Increased weight for height
 Dry skin
 Increased body hair
 Pallor
 Myxedema of the face
 An enlarged thyroid gland
 Proximal muscle weakness
 Delayed relaxation phase of the ankle reflex, and
 Delayed puberty
 Occasionally, acquired childhood hypothyroidism presents
with precocious puberty.
 The enlarged thyroid gland usually is diffuse and non tender;
Sometimes the gland may be firm.
 The onset of acquired childhood hypothyroidism often is very
subtle; in retrospect, it may be evident that signs and
symptoms were present for a longer time, sometimes for 2 to 3

275
 or more years. If previous height measurements are available,
a decline in linear growth from the onset of hypothyroidism will
be evident.

Labo ratory Tests

For new-born screening:
 Initial T4 testing with follow-up TSH testing
 Between 1 and 4 days of age, the normal range for serum
total T4 concentrations is 10 to 22 mcg/dL (128.7 to 283.2
nmol/L).
 Between 1 and 4 weeks of age, the normal range for serum
total T4 concentrations is 7 to 16 mcg/dL (90.1 to 205.9
nmol/L).
 Subclinical hypothyroidism is defined as a normal serum total
or free T4 concentration and a high serum TSH concentration.
 Infants who have subclinical hypothyroidism should be treated
during the first 3 years after birth due to the critical
dependence of the myelinizing CNS on thyroid hormone.

Management
Congenital
 The overall goals of treatment are normal growth and good
cognitive outcome.
 Serum T4 concentrations should be restored rapidly to the
normal range, followed by continued maintenance of
euthyroidism.
 The aim of treatment is to keep the serum T4 or free T4
concentration in the upper half of the normal range adjusted
for age.
 Of note, many commercial laboratories do not provide age-
adjusted normal ranges in their reports.
 In the first postnatal year, serum T4 should be 10 to 16 mcg/dL
and serum freeT4 should be1.4 to 2.3ng/dL.
 The serum TSH concentration should be less than 5 mU/L.
 Oral T4 (levothyroxine) is the treatment of choice.
 Initial dose of 10 to 15 mcg/kg per day, usually 50 mcg/day.

276
 In preterm and other low-birthweight infants, the thyroid
replacement dose should be calculated by using10
to15mcg/kg per day, with the higher end administered to
babies who have low T4 concentrations.
 Only T4 tablets should be used because thyroid suspensions
prepared by individual pharmacists may result in unreliable
dosing.
 Parents should be instructed to crush the T4 tablet and mix It
with a small volume of human milk, formula, or water. Soy
formulas or any preparation containing concentrated iron or
calcium should not be used because they reduce the
absorption of T4.
 The more rapidly T4 concentrations are corrected, the better
the neurologic outcome. Aim to normalise the T4
concentrations within two weeks

Patient follow up
The recommended follow up schedule is as follows;

 At 2 and 4 weeks after the initiation of T4 treatment

 Every1to2monthsduringthefirst6postnatalmonths
 Every3to4monthsbetween6monthsand3yearsofage
 Every 6 to12months thereafter until growth is complete
 2 weeks after any change in dose
 At more frequent intervals when compliance is questioned or
abnormal results are obtained

Acquired
 Childhood hypothyroidism is treated with levothyroxine. (refer
to table below for dosage)
 Treatment should be individualized because the absorption of
T4 and metabolism vary among individuals.
 Serum freeT4 and TSH concentrations should be monitored
periodically, preferably at 3-to 6-month intervals.
 The goal is to keep the serum free T4 concentration at the
mid-normal range and the TSH concentration in the normal
range.

277
 Once the patient is euthyroid, many of the symptoms
disappear.

Dosage of levothyroxine
6 to 12 months 1 to 3 years 3 to 10 10 to 18
Age band
years years
5 to 8 mcg/kg 4 to 6 3 to 5 2 to 4
Dosage
mcg/kg mcg/kg mcg/kg

Refere nces
1. Debra Counts, Surendra K. Varma, (2009); Hypothyroidism in
Children, Pediatrics in Review Vol.30 (7) retrieved from
ttp://pedsinreview.aappublications.org
2. LaFranchi SH, Hanna CE (2005). The thyroid gland and its
disorders. In: Kappy MS, Allen DB, Geffner ME, (ed). Principles
and Practice of Pediatric Endocrinology. Boston, Mass:
Charles C Thomas; 279–356
3. Raine JE, Donaldson MDC, Gregory JW, Savage MO, Hintz R
(2006); Thyroid disorders. In: Practical Endocrinology and
Diabetes in Children. London, United Kingdom: Blackwell;
91–108

4. Selva KA, Harper A, Downs A, Blasco PA, Lafranchi SH (2005);

Neurodevelopmental outcomes in congenital
hypothyroidism: comparison of initial T4 dose and time to
reach target T4 and TSH. J Pediatr. 147:775–780 2.

5. Grant DB, Fuggle P, Tokar S,Smith I. (1992); Psychomotor

development in infants with congenital hypothyroidism
diagnosed by neonatal screening. Acta Med
Austriaca;19(suppl1):54

278
NEONATOLOGY

HYPOXIC ISCHAEMIC ENCEPHALOPATHY (HIE)

Definition
HIE is an acquired syndrome of acute brain injury characterized
by Neonatal Encephalopathy (NE) and evidence of intrapartum
hypoxia.

NE is characterized by an abnormal level of consciousness,

abnormal tone and abnormal primitive reflexes. Abnormal
breathing and seizures may also occur.
Intrapartum hypoxia may be suggested by the presence of one
or more of the following features:
 An acute intrapartum event
 Fetal bradycardia or reduced variability on CTG
 Meconium stained liquor
 Prolonged second stage
 Need for resuscitation at birth for 10 minutes or longer
 a 5-minute Apgar score < 7
 Acidosis on cord or neonatal blood in the first hour of life
(defined as pH < 7 or Base Excess < - 10).

NOTE: The presence of features that suggest intrapartum

hypoxia does not exclude other causes of encephalopathy.

Immediate manage me nt o n admission

 Manage A- airway, B- breathing and C- circulation
 Check glucose, give glucose at 3mls/kg if < 2.6 mmol/L
 Document congenital anomalies
 Document the Thompson HIE score
 If there are signs of encephalopathy:
o ◊ Keep nil by mouth
o ◊ Commence IV fluids at 40 ml/kg/day
o ◊ Apply the aEEG as soon as possible if available

279
If there are clinical or aEEG signs of moderate-severe HIE, refer to
the cooling protocol in the National Neonatal Guidelines –
discuss all cases with a senior clinician.

Init ial invest igations of infants with moderate –

severe HIE s hould include:
 Blood gas
 FBC, CRP, blood culture
 Electrolytes; Sodium, Potassium, Calcium, Magnesium
 Urea, creatinine
NOTE: If history/signs suggest sepsis, perform a septic screen that
includes a lumbar puncture and commence antibiotics

Init ial invest igations of infants with moderate –

severe HIE
Cerebral function monitor, aEEG

 If available, apply aEEG to all infants with signs of HIE as soon

as possible
 Monitor for at least 6 hours if not cooled (preferably 24h). If
cooled, continue until rewarm is complete
 A severely suppressed background persisting for 6 hours
predicts a poor outcome in over 80% and if this persists
beyond 48 hours, despite cooling, the prognosis is almost
invariably poor
 A normal voltage pattern at 48 hours in cooled infants
associated with a good outcome in most infants

Cranial ultrasound

 Performed at least on Day 1 and again at Day 7-14

 Cranial US shows gross anatomy, established damage at birth
and evolving focal or global injury.

MRI

 MRI is the most reliable guide to prognosis and diagnosis

available. It is best done at 7-14 days for optimal prognostic
information.
280
Clinical manageme nt of infants with moderate –
severe HIE
Temperature and Coolin g
 Avoid overheating infants at any stage
 Maintain normothermia
 Refer to separate cooling protocol for details of eligibility and
refer to a facility that is able to cool (Discuss with a senior
clinician).
 It is not necessary to obtain the aEEG result before cooling if
infants have obvious moderate-severe encephalopathy on
clinical grounds
Ventilation

 Aim for normal PaCO2 and oxygen saturation (Targets:

PaCO2 35-45mmHg; Preductal Sats 90-95%)
 If oxygen is needed and respiratory effort is good, nasal CPAP
or nasal cannulae are often adequate
 Ventilate if apnoea or respiratory acidosis with pH < 7.25 and
inform senior clinician
 If ventilated do blood gases 8 hourly or more often if
abnormal

Blood pressure

 Monitor blood pressure and keep it in the normal range (Mean

arterial pressure [MAP] = 40-55)
 Avoid fluid boluses unless hypovolaemia is suspected. Give
fluid bolus of normal saline at 10mls/kg if suspected
hypovolemia
 Metabolic acidosis alone, is not an indication for a fluid bolus
 Treat normovolaemic hypotension with inotropes. Use central
access via UVC or other central line
 An echocardiogram to assess cardiac function can inform
fluid and inotrope management

Haemoglobin

281
 Transfuse if significant anaemia
 Transfusion thresholds:
o Hb < 10g/dL if on CPAP
o Hb < 11g/dL if FiO2 < 0.3; < 12 if FiO2 > 0.3 in neonates on
assisted ventilation
Coagulation management

 Check INR/PTT/Fibrinogen in severely anaemic or bleeding

infants (e.g. subaponeurotic haemorrhage)
 Treat active bleeding with vitamin K (1-2mg/kg IV) and FFP
(15mls/Kg over 30 minutes) – consider cryoprecipitate if poor
response or low fibrinogen

Fluid balance, acidosis and metabolic management

Intrinsic renal injury and SIADH commonly occur

 Initially fluid restrict to 40 ml/kg/24 hours
 Initially use 10% dextrose solution
 Potassium-containing fluid should only be used if urine output
and serum potassium are normal
 Monitor urine output, electrolyte, blood glucose and blood
gases
 Catheterise if urine retention or oliguric (<1 ml/kg/hr)
 Hypocalcaemia and hypomagnesemia should be
anticipated and treated
 Treat Hyponatraemia< 130 mmol/l with fluid restriction if
oliguric – if not oliguric, consider increasing fluids and using
0.9% saline in maintenance fluids
 There is no proven benefit of Sodium Bicarbonate to treat
lactic acidosis
 Monitor plasma glucose
 Increase IV glucose concentration if plasma glucose remains
< 2.6 mmol/l use 12.5% or 15% glucose as an infusion solution
rather than increase volume

Feeding

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 Beware of necrotizing enterocolitis
 Introduce feeds slowly, preferably breast milk (assess suck
reflex)
 Keep nil by mouth on admission then commence 3-hourly
feeds after 12-24 hours:
 Increment by 12-24mls/kg/day during the first 3 days and by
up to 30ml/kg/day thereafter if abdomen exam normal
 Infants with mild encephalopathy who are not cooled can be
fed with higher volumes

Sepsis

 Treat with first line antibiotics as per hospital protocol.

 Discontinue antibiotics at 36 hours if sepsis excluded with
normal CRP at 36-48 hours or follow hospital antibiotic policy.

Seizures

Refer to seizure management guideline

Communication with Parents

 Explain the clinical condition and the potential for other

causes
 Explain the management
 Document the parent’s version of events
 Prepare them for a potential poor outcome if investigations
and signs are suggestive

Clinical neurological assessment

Clinical signs vary with time. There are three stages of

encephalopathy:
 Stage 1(mild): Irritability, increased tone, poor sucking and
exaggerated moro reflex
 Stage 2 (moderate): Lethargy, decreased tone and primitive
reflexes. Often with seizures
 Stage 3 (severe): Stupor or Coma, flaccid tone and seizures
often clinically less apparent Moderately or severely affected
283
 infants typically develop increasingly obvious signs during the
first 48-72 hours. Seizures are often clinically silent.

The Thompson HIE score

The Thompson HIE score should be performed on admission,

before age 5 hours, and daily until normal or age 7 days (up to
10 days if cooled). The maximum score, based on the clinical
signs in the previous 24 hours, is recorded in each category and
then totalled for the day. A score of < 5 in the first 5 hours of life is
associated with a normal aEEG at 6 and 24 hours.

If infants are not cooled:

A peak score of 10 or less during the first 6 days with a score of 0

by day 7 predicts a normal outcome, a score of 11-15 at any
time or a score above 0 on day 7 predicts an abnormal
outcome in 65% and a score of above 15 predicts an abnormal
outcome in 92%.

When to stop resuscitation or offer withdrawal/redirection of care

 Stop resuscitation after 10 minutes of asystole

 Consider stopping resuscitation if no spontaneous respiration
after 20 minutes – first ensure that infant is not hypocarbic and
has not been exposed to maternal opiates/benzodiazepines
 Discuss with senior clinician before stopping resuscitation

Withdrawal of intensive care or ventilation

Should be discussed with the senior clinician and should be

considered (withdrawal may be mandatory if resources are
limited), in the following circumstances:
 Infants who initially met resuscitation discontinuation criteria
but ventilation was continued and there is persisting isoelectric
aEEG and/or coma and persistent apnoea in the absence of
sedative drugs

284
 Infants with severely abnormal aEEG (upper and lower
margins below 10mcV or burst suppression) persisting beyond
24 hours if not cooled or 48 hours if cooled.
NOTE: high dose midazolam or opiates can affect the clinical
and electrical data

 Additional factors such as cranial ultrasound and MRI findings

and multiorgan failure should also be considered.

Follow-up

 Arrange neurodevelopmental follow up at 20 (18-22) weeks

(to follow up with local hospital guidelines)
 Document head circumference and neurological exam at
birth and at discharge
 Document maximum HIE grade and Thompson score
 Plan and discuss the need for long-term anticonvulsant
management (refer to neonatal seizures section).
 Limited consensus exists concerning the duration of long-term
AED therapy and controlling seizures
 Normal examination, in particular a normal nutritive suck at
age 7 days carry a good prognosis.
 An infant who can feed well and visually fix and follow by age
10-14 days can still have significant focal basal ganglia lesions
that may lead to an athetoid type of CP with good
preservation of intellect and head growth
 Infants with adequate feeding by 2-3 week but often slightly
slow visual attention may have significant white matter injury
that will lead to relative microcephaly and some slowness in
learning without a major motor problem.
NOTE: If MRI is done after discharge, ensure that a
developmental appointment is made soon after to discuss
results.

Refere nce
Zambia Neonatal Protocols and Drug Doses 2016

285
PREMATURITY
Delivery of an infant born before 37 completed weeks of
gestation. It is categorized by gestation age or birthweight.

Gestatio n age
Late preterm infant - infant born between 34 and 36 weeks
gestation
Moderately preterm infant - infant born between 32 and 34
completed weeks of gestation

Very preterm infant - infant born between 28 and 32 completed

weeks of gestation
Extreme Preterm infant - born before 28 weeks

Birth weight
 Low birthweight (LBW) - 1500g-< 2500g
 Very low birthweight (VLBW) - 1000g- <1500g
 Extremely low birthweight (ELBW) - <1000g

Diagnosis
By assessing the gestational age at birth by using Ballard scoring
system or foot length (Refer to the Zambia Neonatal Protocols
and Drug Doses).

Management
Routine care
 Airway, breathing, cord and eye care

Thermal protection
 Maintain a set environmental temperature of 25°C
 All infants should be received into a clean, dry pre-warmed
towel
 Infants less than 30 weeks gestation age or 1200g birth weight
should be placed into a plastic bag up to neck. Cover the
head but not the face. DO NOT DRY THESE INFANTS
 Continue to resuscitate with the infant in the plastic bag. Cut
holes in bag for limb or umbilical vascular access if needed

286
 Weigh the infant inside plastic bag
 Keep infant in bag during transfer to neonatal unit
 Remove plastic bag once stabilized in the NICU in an
incubator
 Use KMC for the stable preterm infant (Refer to National KMC
Guidelines)
 Document temperature on arrival in NICU and after 1 hour

Fluid requirements

The expected early postnatal weight loss is higher in preterm

infants and they may lose up to 15% of their body weight.
Give 10% dextrose in the first 36 – 48 hours, 5% dextrose is suitable
for ELBW infant because it has a low solute load which can easily
be handled by the immature kidneys. After 36 - 48 hours change
to either ¼ strength Darrow’s solution in 10% dextrose or 10%
Neonatalyte.

Initiate fluids as below:

Birth Weight(g) Fluids Day 1 Glucose

(ml/kg/day) (mg/kg/min)
<1000 5% Dextrose 90 3.2
1000 – 1199 10% Dextrose 80 5.6
1200 – 1499 10% Dextrose 70 4.2
>1500 10% Dextrose 60 4.2

 Maintain glucose between 2.6 – 7mmol/l

 Increase total fluid intake daily in increments of 10 – 20
ml/kg/day, dependent on urine output, weight and serum
sodium

Enteral feeding
 Breast milk feeding must be encouraged for all infants.
 Donor breast milk is preferable to formula for preterm infants
unable to access mother’s own breast milk.
 Fluid and enteral intake prescriptions should be individualized
for sick infants and infants with risk factors or feed intolerance.
287
≥ 1 5 00 G OR ≥ 32 Weeks
o In the absence of mother’s own milk, consider preterm
formula
o Start on bolus feeds 2 -3 hourly at 60ml/kg on day 1
o Increase to 75, 100, 125, 150ml/kg/d from D2-D5 as feeds
are tolerated

< 1 5 00 G OR 32 Weeks
o Start with bolus tube feeds (expressed breast milk (EBM))
o Orogastric tubes are preferable to nasogastric tube
o Start milk on D1 at 12-24 ml/kg/d
o Increase feeds daily by 24 ml/kg/day
o Consider continuous feeds if prolonged significant
feeding intolerance – discuss with senior clinician.
o Syringes should be changed every 4 hours for all feeds
given via a syringe driver
o Continue until a rate of 10ml/hr or weight of 1 200 g is
achieved before challenging with 2-hourly bolus feeds
o Stop supplemental IV fluids when an enteral intake of
150 ml/kg/day is achieved (consider individual baby
tolerance)
o Increase enteral volume incrementally to 180-200
ml/kg/day
o Add supplements and breast milk fortifier according to
guidelines
o The feeding tube must be changed weekly, and the
administration set three times a week
o Initiate TPN for confirmed prolonged feeding intolerance
> 3 days and for confirmed cases of NEC (See TPN
Protocol in the Zambia Neonatal Protocols)

Prete rm Supple ments

288
Preterm infants on full feeds or feeds at 150ml/kg should be given
the following supplements for 6 months:
 1500g – 2500g
o Multivit 0.6ml PO daily
o Iron syrup or drops 0.6ml PO from day 28 of life

 < 1500g
o Introduce Multivit 0.3ml PO daily 48 hours after
establishing full feeds
o Iron syrup or drops 0.2ml PO from day 28 of life
o Add human milk fortifier to breastmilk 24 hours after
establishing full feeds

Infection
Treatment and /or prevention (See section on sepsis in neonates)

289
RESPIRATORY DISTRESS SYNDROME
 RDS is a problem often seen in premature babies
 The condition makes it hard for the baby to breathe
 Occurs in infants whose lungs have not yet fully developed
due to a lack of surfactant which helps the lungs fill with air
and keeps the air sacs from deflating.

Clinical Features
Appear within minutes of birth, however, they may not be seen
for several hours. These may include:
 Cyanosis
 Apnea
 Nasal flaring
 Rapid breathing
 Shallow breathing
 Grunting

Investigations
 Blood gas analysis shows low oxygen and excess acid in the
body fluids
 Chest x-ray shows a "ground glass" appearance to the lungs
that is typical of the disease. This often develops 6 to 12 hours
after birth.
 Lab tests help to rule out infection as a cause of breathing
problems

Treatme nt
 Give warm, moist oxygen
 Monitor carefully to avoid side effects from too much oxygen
 Give surfactant intra-tracheal
 Start CPAP or HFNC
 Intubate and ventilate for preterm infants who are not coping
on CPAP or remain apnoeic

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NECROTISING ENTEROCOLITIS (NEC)
 NEC is a life-threatening condition that affects the bowel of
infants, the majority of whom are preterm
 Patients are severity ill, with an acute abdomen
 NEC should be considered in any preterm infant with
abdominal distention
 Etiology is multifactorial; prematurity is the biggest
independent risk factor but infection, hypoxia, feeding
regimens and type of feed (hyperosmolar formula),
polycythemia, PDA, indomethacin may play a role
 The use of breast milk is associated with a lower incidence of
NEC.

Clinical features
 NEC can occur in the first few days of life in term patients, but
more commonly from the second week onward in preterm
infants.
 Onset can be insidious but patients with a rapid onset
deteriorate quickly
 Lethargy, abdominal distension, absent bowel sounds, bloody
stools, vomiting and discolored abdominal wall are the
cardinal signs
 Other signs of illness are tachycardia, respiratory distress and
poor perfusion may be present.

Modified Bell’s st aging

Stage 1. Suspected NEC: clinically ill.

Abdominal X-ray: normal to mild distension of bowel loops

Stage 2. Definite NEC: mild or moderate systemic illness, absent
bowel sounds, abdominal tenderness, metabolic acidosis, low
platelets.
Abdominal X-ray: pneumatosis intestinalis or portal venous gas.
Stage 3. Advanced NEC: severely ill, marked distension, signs of
peritonitis, hypotension metabolic and respiratory acidosis, DIC.

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Abdominal X-ray: pneumatosis intestinalis, portal venous gas or
pneumoperitoneum

Management
 Resuscitate: Support breathing and intubate if required.
 Assess circulatory status; apply fluid and inotropic support
judiciously
 Stop enteral feeds immediately. Insert naso-/orogastric tube,
leave on free drainage, monitor gastric output
 Strict input-output monitoring
 Investigations: Abdominal X-ray, FBC, CRP, blood culture.
Consider U&E, ABG and INR/PIT.
 Start broad-spectrum antibiotics including adequate gram-
negative cover
 Serial abdominal examination is important

Stage 1

 Reassess abdomen in 24-48 hours. Restart small volume feeds

cautiously if examination is normal

Stage 2 and 3
 Keep NPO for 5-10 days. Start total parental nutrition
preferably via a deep percutaneous venous line by 48 hours
NPO.
 Patients with advanced disease may require repeat FBC, and
clotting profile. Blood products (i.e. packed RBC, platelets or
FFP) should be transfused if anaemic and/or DIC present
(Refer to transfusion protocol in Zambia Neonatal Protocols).
 Surgery: operative intervention may be indicated if:
pneumoperitoneum, fixed bowel loop, abdominal mass
palpable or persistent metabolic acidosis. Paediatric surgeons
should be consulted after initial stabilization.
 DO NOT GIVE INDOMETHACIN TO AN INFANT with suspected
or definite NEC
 Peritoneal drainage can be used in patients who are too
unstable for surgery or in whom ventilation is difficulty due to

292
 abdominal fluid. Consult with Paediatric surgeon present.
Restart feeds slowly (20-30ml/kg/day)
 Continue antibiotics until septic markers are normalized

Prevent ive me asures

 Start small volume feeds early to stimulate GIT mucosal
development
 Cautious advancement of feeds in small premature infants
 Do not increase feeds if infant has gastric residual of 50% or
more
 Breast milk appears protective

High risk patients

In the presence of the following risk factors, feeds should be
initiated more cautiously in discussion with a senior clinician:
 Neonatal encephalopathy
 Preterm and metabolic acidosis at birth
 Cardiorespiratory instability

Other complications associated with prematurity are discussed

in the Zambia Neonatal Protocols and Drug doses:
 Apnoea of Prematurity
 IVH
 BPD
 ROP
 PDA

Refere nce:
Zambia Neonatal Protocols and Drug Doses 2016

293
SEPSIS IN NEONATES

Definition
Neonates are particularly susceptible to bacterial sepsis. The
highest incidence being in the very low birth weight infants.

Early-onset sepsis
 < 72 hours after birth
 Results from vertical exposure to bacteria before and during
birth

Late-onset sepsis
 > 72 hours after birth
 Mostly from organisms acquired by nosocomial transmission
 May also be caused by community acquired organisms

Risk factors
Early-onset sepsis

 Preterm infant
 Prolonged rupture of membranes > 18 hours
 Maternal fever in labour (>38 C)
 Chorioamnionitis
 Maternal colonization with Group B Streptococcus

Late-onset sepsis

 Preterm infant
 Indwelling catheters or tracheal tube
 Prolonged antibiotics
 Damage to skin

Clinical features
 Usually non-specific
 Altered behaviour or responsiveness
 Apnoea and bradycardia

294
 Respiratory distress - increase in oxygen
requirement/respiratory support
 Poor feeding, poor suck, vomiting, abdominal distension
 Hypoglycaemia or hyperglycaemia
 Fever, hypothermia or temperature instability
 Cyanosis or poor colour
 Poor perfusion (CRT>3sec; mottling)
 Hypotension
 Tachycardia
 Circulatory collapse or shock
 Irritability, inactivity, lethargy
 Seizures
 Hypotonia
 Jaundice
 Rash

Meningitis
 Tense or bulging fontanelle
 Head retraction (Opisthotonus)

Investigations
 Full Blood count
 C-reactive protein
 Blood culture
 Lumbar puncture – if blood culture positive or clinical features
of meningitis
 CXR – if indicated
 Tracheal aspirate
 Coagulation screen
 Blood gas
 Placental tissue culture and histopathology

Interpretatio n of labo ratory investigat ions

Features suggestive of sepsis:
295
 Neutropenia or neutrophilia
 Increased ratio of immature(bands): total neutrophils
 Thrombocytopaenia
 Positive blood culture
 Raised CRP (>10 mg/L)

Treatme nt
 Supportive care
 Start antibiotics – antibiotic choice depends on local
incidence and practice

Duration of antibiotics
 Blood culture negative, CRP remains normal and no clinical
signs of infection – Stop antibiotics at 36-48 hours
 Blood culture negative but CRP raised – treat as infected
 Blood culture positive – treat until clinical improvement and
CRP has returned to normal (7-10 days)
 Meningitis – 14-21 days

NOTE: In the absence of proven sepsis there is no role for a

course of antibiotics

Antibiotic policy for suspected se psis

Antibiotics policy is determined by considering the organism
known to have caused infection in the neonatal unit/nursery in
the last 6 months. This policy should be regularly reviewed and
revised as necessary. A disciplined and consistent approach to
antibiotic usage is necessary to provide optimal broad-spectrum
cover in suspected sepsis and to limit the emergence of resistant
organisms in areas of high usage.

Proposed antibiotics
Decide according to unit cultures and sensitivities
Early onset sepsis (< 72hrs of age): Penicillin G and gentamicin
Late onset sepsis (> 72hrs of age):

 First line Penicillin/Cloxacillin and gentamicin/ amikacin

296
 Second line antibiotics: Cefotaxime and cloxacillin
 Third line: Ciprofloxacin/Meropenem
 Meropenem should be used if meningitis or NEC is suspected
 Vancomycin only if the patient has central lines in place and
is at risk of Staphylococcus aureus sepsis.

Once started, the duration of treatment should be tailored to

clinical circumstances. If cultures come back normal, laboratory
indices are normal and the patient no longer shows signs of
sepsis, stop antibiotics after 36 – 48 hours. In the absence of
proven sepsis, there is no place for “a course” of antibiotics.

Criteria for commencing antibiotics in a baby with

risk o f having an early onset infection

1. Absolute indication for empiric antibiotic treatment

o Maternal invasive bacterial infection requiring antibiotics
(eg: septicaemia) – suspected or confirmed (NB: Not
prophylaxis)
o Confirmed or suspected infection in twin
o Respiratory distress starting more than 4 hrs after birth
o Mechanical ventilation in a term baby
o Seizures
o Signs of shock
o Home delivery

2. Start antibiotics if two or more are present of:

Antenatal
o Preterm birth following spontaneous labour <37 weeks or
Prelabour ROM
o ROM ≥18 hours
o Maternal fever ≥ 38ºC or chorioamnionitis

Postnatal

o Altered behavior/tone/responsiveness

297
 o Feeding difficulties (eg. Poor feeding in a term baby) or
intolerance
o Respiratory distress
o Apnoea
o Abnormal heart rate (bradycardia or tachycardia)
o Altered glucose homeostasis (hypo/hyperglycaemia)
o Metabolic acidosis
o Temperature abnormality >38º or < 36º not explained by
environmental factors

NOTE: If only one risk factor is present, consider

observation for 24 hrs

Refere nce
Zambia Neonatal Protocols and Drug Doses 2016

298
NEONATAL SEIZURES

Definition
An abnormal synchronous electrical discharge of a group of
neurons in the central nervous system.

Status epilepticus: continuous seizures lasting 30 minutes or

recurrent seizures occupying 50% of the EEG recording for at
least 60 minutes.

Clinical manifest atio ns

Clinical manifestation may be:
 Absent
 Subtle: eye deviation, eyelid fluttering, bucco-lingual
movement or pedaling of arms and legs
 Focal: tonic or clonic
 Generalised: multifocal rhythmic jerking, generalized posturing
or myoclonic

Import ant causes

Brain damage: Hypoxia – ischaemia, bleeding, infarction, oedema
Brain malformations
Meningitis or encephalitis: Acute: (bacterial or viral) or Chronic: (viral, syphilis)
Bilirubin encephalopathy
Biochemical: Hypoglycaemia, hypomagnesaemia
Hypernatraemia, hyponatraemia
Hyperammonaemia, pyridoxine dependency, other
Inborn errors of metabolism
Drug withdrawal Maternal opiate or cocaine abuse
Iatrogenic: Air embolism
Familial: Fifth day fits (benign)

Diagnosis
 History: family, pregnancy, birth, clinical course
 Confirm seizure and monitor response to treatment using aEEG
if available
 Measure serum glucose, magnesium, calcium and sodium
299
 Do a lumbar puncture if sepsis is suspected
 Head ultrasound may be diagnostic if intracranial bleed,
structural abnormality or ventriculitis is present
 Consider inborn errors or metabolism if other causes are not
obvious
 Measure serum lactate, ammonia and amino acid and urine
organic acids. Discuss with the consultant need for CSF
lactate and glycine levels. Note serum and CSF glycine levels
have to be done simultaneously.
 Discuss and arrange with the laboratory before taking any
samples.

Treatme nt
 Treat electrolyte and glucose abnormalities and sepsis
 Ensure adequate ventilation and perfusion. Commence
CFM/Brain monitor
 Treat seizures (clinical and/or electrical) with anticonvulsants if
they are recurrent or last more than 3 minutes.

Anticonvulsant tre atment algo rithm fo r term infant s

300
 Phenobarbitone 20mg/kg IV infused over 10 min

Seizures persist

Repeat phenobarbitone dose

Seizures persist

Midazolam/(Diazepam)
Refer to formulary for dose and weaning procedure

Seizures persist

Lignocaine* or phenytoin
Refer to formulary for dose: NB: use lower dose in cooled infants

Seizures persist

Pyridoxine 100mg IV/IM/PO

Restart Midazolam infusion, or
Clonazepam 0.1mg/kg slow bolus, or
Lorazepam 0.05 - 0.1 mg/ IV/IM/PO#

 If IV Phenobarbitone is not available, use midazolam,

clonazepam or Lorazepam as first line. In addition, consider
treating with oral phenobarbitone as crushed tablets.
 If Lignocaine is not available, use Phenytoin as second line.
 Do not use Lignocaine and Phenytoin in the same patient
within 72 hours of each other.
 #Lorazepam: IV use, dilute to 0.1mg/ml. IM use (emergency)
dilute to 1mg/ml. Use sugar free suspension for oral use.

NOTE: Ensure the lignocaine vial is suitable for IV use. Lignocaine

infusions must be checked by 2 senior staff before
commencement
In preterm infants follow the same algorithm but use phenytoin
as second line instead of lignocaine

Treatme nt co nsideratio ns

301
1. When the seizures are controlled, keep the newborn in
Phenobarbitone maintenance at 3-5 mg/Kg/day.
NOTE: In case of excessive sedation do not discontinue
Phenobarbitone (probably the drowsiness is secondary to
the underlying condition) and evaluate reduction
of Phenobarbitone at lower maintenance dose with close
clinical monitoring.
2. In case of acute symptomatic seizures resolved by
correction of the underlying etiology and not associated
with increased risk of brain injury, AEDs can be discontinued
gradually.
3. If the infant is taking medication and seizures recur, increase
the dose back to levels at which no seizures occurred and
ask for neurological evaluation.
4. Generally, patients who had neonatal seizures that required
recommencement of anticonvulsants/continued AEDs;
should be discharged on maintenance phenobarbital and
followed up monthly at least until 3 months of age. The
choice to tapper down Phenobarbitone after 1 month
should be evaluated case by case considering risk factors
for recurrence of seizures.
5. Paediatric neurology evaluation and EEG at 1- 3 month for
infant at increase risk of epilepsy should be requested where
available.

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NEONATAL JAUNDICE

Definition
 Between 50-60% of normal newborns become clinically
jaundiced in the first week of life
 Jaundice may be physiological or pathological

Physiological jaundice is due to:

 Increased production of bilirubin (large erythrocyte mass with
shortened life span)
 Decreased hepatic excretion of bilirubin (low hepatocyte
ligandin level, low glucoronyl transferee activity)
 Increased entero-hepatic circulation of bilirubin (high
intestinal B-glucoronidase levels decreased intestinal motility)
 Breast feeding jaundice is thought to be due to feeding
problem, which lead to a decreased intake of milk, increased
entero-hepatic circulation and sometimes dehydration
 Breast milk jaundice is diagnosed in clinically well breastfed
infant who remains jaundiced for several weeks following
physiological jaundice. The mechanism is unknown. It is
thought that breast milk glucoronidase leads to increased
absorption of unconjugated bilirubin via increased entero-
hepatic circulation. Diagnosis is by exclusion. Breastfeeding
may be continued.

Pathological jaundice
may result from an increased unconjugated or conjugated
fraction or both.

Red flags (features of pathological jaundice plus)

 Mother Rh negative
 Mother blood group O. If known check total serum bilirubin
(TSB) at 6 hours post-delivery
 Baby Coombs positive
 Anaemia
 Evidence of haemolysis
 Preterm

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 Acidosis, hypoglycaemia, hypoxaemia, hypothermia
 Features of kernicterus
 Bilirubin levels not decreasing despite effective phototherapy,
(i.e. 17 – 34 µmol/l within 4 – 6 hours)
 Family history of pathological jaundice

Features Physiological jaundice Pathological jaundice

Clinical onset of > 36 hours ≤ 24 hours

jaundice (after birth)

Duration of jaundice Term <10days Term >Day 10-14

Preterm <21 days Preterm > Day 21

Peak TSB Term Day 3 Early or late

(days after birth) Preterm Day 5 – 7

Peak TSB < 275 µmol/l > 272µmol/l

Rise in TSB per 6 hours >50 µmol/l

Conjugated serum Only unconjugated >34 µmol/l

bilirubin fraction increased

Evidence for No Yes/No

haemolysis

Underlying illness No Yes/No

Hepatomegaly No Yes/No

Pale stool/dark urine No Yes/No

Management
Early onset jaundice (within 24 hours) – caused by haemolytic
disease of new born (HDN) (ABO or rhesus):
 Check mother’s blood group
 If the mother’s blood group is O, ABO likely. Do following
investigations:
o Infants blood group and Coombs

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 o FBC and peripheral smear
o Check TSB 3 hourly
o Start phototherapy
o In rare cases look for other causes for haemolysis

Jaundice after 24 hours

 Exclude blood incompatibility
 Exclude obvious infection or extravasated blood/bruising
 Check feeding and infant’s weight to exclude breastfeeding
jaundice
 Exclude polycythaemia
 Often a cause will not be found; in term infants thought to be
an exaggeration of physiological hyperbilirubinaemia and in
preterm infants due to the immaturity of the conjugation
mechanism
 Treatment will depend on the level of the TSB which should be
checked daily

Other modes of assessment

Transcutaneous bilirubin (TCB) screening

 TCB is a screening tool to select infants who require formal

total serum bilirubin (TSB)levels
 TCB screening decreases the number of heel pricks and
readmissions for phototherapy
 Do not rely on TCB monitoring in severe hyperbilirubinaemia
 TCB should not be used within 24 hours of phototherapy or an
exchange transfusion
 Localised oedema and decreased tissue perfusion affects
accuracy of TCB measurements
 Technique:
o Ensure all staff are properly trained on the TCB device
o Calibrate the TCB device daily in accordance with the
manufacturer’s specification
o Preferred sites are inter-scapular (for infants < 35 weeks),
forehead or sternum
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 o Measurements should not be made over bruised skin,
areas covered by hair or birthmarks
o Measure TCB by gently pressing the tip of the device
over the examination site then press the trigger button
until the device indicates the measurement is complete
o Take 3 - 5 measurements (device dependent) from the
examination site, ensuring consistent placement of the
tip and amount of pressure applied
o If the TCB reading is within 20 μmol/L of the
phototherapy line do TSB

Kramer’s rule

In a resource limited country like Zambia, laboratory diagnostics

may not be available in a timely manner. Clinical examination
(e.g Kramers rule) can be used in making the decision to start
phototherapy (It should be known that clinic estimation of
jaundice is very unreliable, especially in dark skinned infants)
Kramer’s rule entails visual inspection in natural light. Depth of
jaundice progresses from head to toe as the level of bilirubin rises
a follows:
Zone Baby’s body area Approximate Diagram
bilirubin level
1 Head and neck 6 mg/dl
(100 µmol/L)
2 Upper body 9 mg/dl
(chest) (150 µmol/L)
3 Lower body, below 12 mg/dl
the belly button (200 µmol/L)
and upper thighs
and arms
4 Lower legs and 15 mg/dl
forearms (250 µmol/L)
5 Hands and feet >15 mg/dl
(>250 µmol/L)

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If you can assess the level of jaundice, any jaundice from the
chest and below, you must initiate phototherapy. (Initiate
phototherapy for any jaundice from the chest and below)

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Treatme nt
Phototherapy
High intensity phototherapy (PT) reduces total serum bilirubin
(TSB) and decreases exchange transfusions.

Indications

 See phototherapy guideline charts

 Indicated for unconjugated hyperbilirubinaemia
 In the absence of TCB to help in decision making, DO NOT
wait for TSB results before starting phototherapy (Kramer
classification can be used if possible)
 A low threshold to start phototherapy in high risk infants (e.g.
with sepsis, HIE etc)

Contraindications

 Congenital porphyria or a family history of porphyria

 Concurrent therapy with metalloporphyrin haem oxygenase
inhibitors
 Concurrent use of drugs or agents that are photosensitisers

Technique
 Intensive PT is defined as light wavelengths between 430-
490nm, delivered spectral irradiance of 30µW/cm2/nm or
higher to the greatest exposed body surface
 Position the PT unit approximately 40cm from the infant
 Measure the irradiance of phototherapy units periodically with
the use of a photoradiometer

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 PT units require adequate ventilation. Do not cover with
blankets

Care of the infant receiving phototherapy

 Monitor temp and ensure adequate fluid intake

 Cover the eyes with gauze pads and place infant naked
under lights (nappy un tied)
 Remove the eye pads during feeds and observe for
conjunctivitis
 Turn infant every 2-3 hours
 In severe jaundice check the TSB 3 hourly
 Visual assessment of jaundice is unreliable once the infant is
under phototherapy

Efficacy of PT
 Successful PT should produce a decline in TSB of 17-34 µmol/L
within 4-6 hours and TSB should continue to fall
 Stop PT if TSB ≥ 50µmol/L below the PT line

Complications

Rashes, “bronzing”, loose stools, dehydration, hypothermia,

hyperthermia and separation for mother

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PROLONGED NEONATAL JAUNDICE

Defined as jaundice lasting >14 days in term infant >21 days in a

preterm infant
Determine whether it is unconjugated or conjugated
hyperbilirubinaemia.

Unconj ugated hype rbilirubinaemia

 Determine whether or not the baby is breastfed
 Collect urine for MCS and reducing substances to exclude
galactosaemia
 Check liver enzymes
 Exclude hypothyroidism
 Exclude haemolysis, check reticulocytes and Hb
 Hereditary enzyme defects such as Gilbert’s and Crigler –
Najjar syndromes are rare

Conjugated hyperbilirubinaemia
 History and examination
 Liver function tests and cholesterol
 Examine stools daily. Acholic (white) stools require urgent
referral to exclude biliary atresia
 Exclude infective causes
 Exclude metabolic causes
 Exclude genetic conditions

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Causes of conjugated hyperbilirubinaemia

Causes

Infective Viral: Hepatitis A, B, CMV, HIV, rubella, herpes simplex

Bacterial: Syphillis, septicaemia, UTI

Protozoal: Toxoplasmosis gondii

Biliary Biliary atresia, choledochal cyst, Alagille’s syndrome, bile

plugs, cystic fibrosis

Metabolic/ Alpha 1-antitrypsin, tyrosinaemia type 1, galactosaemia,

genetic wilson’s disease, hypothyroidism, hypopituitarism, familial
intrahepatic cholestasis, rotor and Dubin-Johnson
syndrome

Drug/toxins TPN

Autoimmune Autoimmune hepatitis, sclerosing cholangitis

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Diagnostic workup for conjugate d
hyperbilirubinaemia

Investigation Test Date Results

Taken
FBC Hb, WCC, platelets,
reticulocytes
Haemotology Coagulation INR, PTT, fibrinogen
Blood group
Coombs
U&E Na, K,urea, Creatinine
TSB, conjugated TSB, conjugated bilirubin
bilirubin
Biochemistry Liver functions AST/ALT, ALP, GGT
CMP Ca, Mg, PO4, Albumin
Cholesterol*
Glucose
Blood culture
Microbiology
Urine culture
IgM Rubella, HSV
Urine CMV
Virology
Hepatitis A, B, C
HIV
Urine reducing
substances
GAL-1-PUT*
alpha 1-antitrypsin*
Plasma amino
Metabolic
acids*
Urine amino acids*
Urine organic
acids*
Sweat test*
Endocrine Thyroid functions
Abdominal U/S
CXR/spinal X-ray*
Radiology
Radio-isotope
scan*
Histology Liver biopsy*
Stool
Other
Ophthalmology*

*Second line investigations, discuss with senior clinician

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